A constituent of STRIATED MUSCLE and LIVER. It is an amino acid derivative and an essential cofactor for fatty acid metabolism.
An enzyme that catalyzes reversibly the conversion of palmitoyl-CoA to palmitoylcarnitine in the inner mitochondrial membrane. EC 2.3.1.21.
An enzyme that catalyzes the formation of O-acetylcarnitine from acetyl-CoA plus carnitine. EC 2.3.1.7.
Acyltransferases in the inner mitochondrial membrane that catalyze the reversible transfer of acyl groups from acyl-CoA to L-carnitine and thereby mediate the transport of activated fatty acids through that membrane. EC 2.3.1.
An acetic acid ester of CARNITINE that facilitates movement of ACETYL COA into the matrices of mammalian MITOCHONDRIA during the oxidation of FATTY ACIDS.
An enzyme that catalyzes the HYDROXYLATION of gamma-butyrobetaine to L-CARNITINE. It is the last enzyme in the biosynthetic pathway of L-CARNITINE and is dependent on alpha-ketoglutarate; IRON; ASCORBIC ACID; and OXYGEN.
A coenzyme A derivative which plays a key role in the fatty acid synthesis in the cytoplasmic and microsomal systems.
A family of proteins involved in the transport of organic cations. They play an important role in the elimination of a variety of endogenous substances, xenobiotics, and their metabolites from the body.
A long-chain fatty acid ester of carnitine which facilitates the transfer of long-chain fatty acids from cytoplasm into mitochondria during the oxidation of fatty acids.
A fatty acid coenzyme derivative which plays a key role in fatty acid oxidation and biosynthesis.
Enzymes from the transferase class that catalyze the transfer of acyl groups from donor to acceptor, forming either esters or amides. (From Enzyme Nomenclature 1992) EC 2.3.
A naturally occurring compound that has been of interest for its role in osmoregulation. As a drug, betaine hydrochloride has been used as a source of hydrochloric acid in the treatment of hypochlorhydria. Betaine has also been used in the treatment of liver disorders, for hyperkalemia, for homocystinuria, and for gastrointestinal disturbances. (From Martindale, The Extra Pharmacopoeia, 30th ed, p1341)
S-Acyl coenzyme A. Fatty acid coenzyme A derivatives that are involved in the biosynthesis and oxidation of fatty acids as well as in ceramide formation.
Pentanoic acid, also known as valeric acid, is a carboxylic acid with a 5-carbon chain (C5H10O2), having a distinctive pungent and rancid odor, found in some animals' sweat, certain foods, and produced through wood fermentation.
Coenzyme A is an essential coenzyme that plays a crucial role in various metabolic processes, particularly in the transfer and activation of acetyl groups in important biochemical reactions such as fatty acid synthesis and oxidation, and the citric acid cycle.
Mitochondria in hepatocytes. As in all mitochondria, there are an outer membrane and an inner membrane, together creating two separate mitochondrial compartments: the internal matrix space and a much narrower intermembrane space. In the liver mitochondrion, an estimated 67% of the total mitochondrial proteins is located in the matrix. (From Alberts et al., Molecular Biology of the Cell, 2d ed, p343-4)
A condition due to deficiency in any member of the VITAMIN B COMPLEX. These B vitamins are water-soluble and must be obtained from the diet because they are easily lost in the urine. Unlike the lipid-soluble vitamins, they cannot be stored in the body fat.
Organic compounds that include a cyclic ether with three ring atoms in their structure. They are commonly used as precursors for POLYMERS such as EPOXY RESINS.
Organic, monobasic acids derived from hydrocarbons by the equivalent of oxidation of a methyl group to an alcohol, aldehyde, and then acid. Fatty acids are saturated and unsaturated (FATTY ACIDS, UNSATURATED). (Grant & Hackh's Chemical Dictionary, 5th ed)
The metabolic substances ACETONE; 3-HYDROXYBUTYRIC ACID; and acetoacetic acid (ACETOACETATES). They are produced in the liver and kidney during FATTY ACIDS oxidation and used as a source of energy by the heart, muscle and brain.
A large lobed glandular organ in the abdomen of vertebrates that is responsible for detoxification, metabolism, synthesis and storage of various substances.
A naturally occurring metabolite of HISTIDINE that has antioxidant properties.
Lengthy and continuous deprivation of food. (Stedman, 25th ed)
BUTYRIC ACID substituted in the beta or 3 position. It is one of the ketone bodies produced in the liver.
Errors in the metabolism of LIPIDS resulting from inborn genetic MUTATIONS that are heritable.
A form of encephalopathy with fatty infiltration of the LIVER, characterized by brain EDEMA and VOMITING that may rapidly progress to SEIZURES; COMA; and DEATH. It is caused by a generalized loss of mitochondrial function leading to disturbances in fatty acid and CARNITINE metabolism.
A chemical reaction in which an electron is transferred from one molecule to another. The electron-donating molecule is the reducing agent or reductant; the electron-accepting molecule is the oxidizing agent or oxidant. Reducing and oxidizing agents function as conjugate reductant-oxidant pairs or redox pairs (Lehninger, Principles of Biochemistry, 1982, p471).
A group of 16-carbon fatty acids that contain no double bonds.
Genetically identical individuals developed from brother and sister matings which have been carried out for twenty or more generations or by parent x offspring matings carried out with certain restrictions. This also includes animals with a long history of closed colony breeding.
Salts and esters of the 16-carbon saturated monocarboxylic acid--palmitic acid.
Electron-dense cytoplasmic particles bounded by a single membrane, such as PEROXISOMES; GLYOXYSOMES; and glycosomes.
Elevated level of AMMONIA in the blood. It is a sign of defective CATABOLISM of AMINO ACIDS or ammonia to UREA.
Hydrazines substituted by one or more methyl groups in any position.
A toxic thiol mercury salt formerly used as a diuretic. It inhibits various biochemical functions, especially in mitochondria, and is used to study those functions.
Derivatives of caprylic acid. Included under this heading are a broad variety of acid forms, salts, esters, and amides that contain a carboxy terminated eight carbon aliphatic structure.
Mitochondria of skeletal and smooth muscle. It does not include myocardial mitochondria for which MITOCHONDRIA, HEART is available.
Salts and esters of hydroxybutyric acid.
Contractile tissue that produces movement in animals.
A fibric acid derivative used in the treatment of HYPERLIPOPROTEINEMIA TYPE III and severe HYPERTRIGLYCERIDEMIA. (From Martindale, The Extra Pharmacopoeia, 30th ed, p986)
The rate dynamics in chemical or physical systems.
A flavoprotein oxidoreductase that has specificity for medium-chain fatty acids. It forms a complex with ELECTRON TRANSFERRING FLAVOPROTEINS and conveys reducing equivalents to UBIQUINONE.
Physiological processes in biosynthesis (anabolism) and degradation (catabolism) of LIPIDS.
The muscle tissue of the HEART. It is composed of striated, involuntary muscle cells (MYOCYTES, CARDIAC) connected to form the contractile pump to generate blood flow.

Cardiomegaly in the juvenile visceral steatosis (JVS) mouse is reduced with acute elevation of heart short-chain acyl-carnitine level after L-carnitine injection. (1/1186)

The long-term administration of L-carnitine was very effective in preventing cardiomegaly in juvenile visceral steatosis (JVS) mice, which was confirmed by heart weight as well as the lipid contents in heart tissue. After i.p. injection of L-carnitine, the concentration of free carnitine in heart remained constant, although serum free carnitine level increased up to 80-fold. On the other hand, a significant increase in short-chain acyl-carnitine level in heart was observed. These results suggest that increased levels of short-chain acyl-carnitine, not free carnitine, might be a key compound in the protective effect of L-carnitine administration in JVS mice.  (+info)

Mutations in the organic cation/carnitine transporter OCTN2 in primary carnitine deficiency. (2/1186)

Primary carnitine deficiency is an autosomal recessive disorder of fatty acid oxidation caused by defective carnitine transport. This disease presents early in life with hypoketotic hypoglycemia or later in life with skeletal myopathy or cardiomyopathy. The gene for this condition maps to 5q31.2-32 and OCTN2, an organic cation/carnitine transporter, also maps to the same chromosomal region. Here we test the causative role of OCTN2 in primary carnitine deficiency by searching for mutations in this gene in affected patients. Fibroblasts from patients with primary carnitine deficiency lacked mediated carnitine transport. Transfection of patient's fibroblasts with the OCTN2 cDNA partially restored carnitine transport. Sequencing of the OCTN2 gene revealed different mutations in two unrelated patients. The first patient was homozygous (and both parents heterozygous) for a single base pair substitution converting the codon for Arg-282 to a STOP codon (R282X). The second patient was a compound heterozygote for a paternal 1-bp insertion producing a STOP codon (Y401X) and a maternal 1-bp deletion that produced a frameshift creating a subsequent STOP codon (458X). These mutations decreased the levels of mature OCTN2 mRNA and resulted in nonfunctional transporters, confirming that defects in the organic cation/carnitine transporter OCTN2 are responsible for primary carnitine deficiency.  (+info)

Mutations of OCTN2, an organic cation/carnitine transporter, lead to deficient cellular carnitine uptake in primary carnitine deficiency. (3/1186)

Systemic primary carnitine deficiency (CDSP, OMIM 212140) is an autosomal recessive disease characterized by low serum and intracellular concentrations of carnitine. CDSP may present with acute metabolic derangement simulating Reye's syndrome within the first 2 years of life. After 3 years of age, patients with CDSP may present with cardiomyopathy and muscle weakness. A linkage with D5S436 in 5q was reported in a family. A recently cloned homologue of the organic cation transporter, OCTN2, which has sodium-dependent carnitine uptake properties, was also mapped to the same locus. We screened for mutation in OCTN2 in a confirmed CDSP family. One truncating mutation (Trp132Stop) and one missense mutation (Pro478Leu) of OCTN2 were identified together with two silent polymorphisms. Expression of the mutant cDNAs revealed virtually no uptake activity for both mutations. Our data indicate that mutations in OCTN2 are responsible for CDSP. Identification of the underlying gene in this disease will allow rapid detection of carriers and postnatal diagnosis of affected patients.  (+info)

Study on propionyl-L-carnitine in chronic heart failure. (4/1186)

AIMS: In patients with chronic heart failure, fatigue is independent of haemodynamic and neuroendocrine changes and possibly may be due to impaired muscle metabolism. Propionyl-L-carnitine, a carnitine derivative, was shown in previous studies to improve muscle metabolism. The objective of this study was to evaluate the effect of propionyl-L-carnitine on exercise capacity in mild moderate chronic heart failure patients, treated with ACE inhibitors and diuretics. METHODS AND RESULTS: This was a phase III, double-blind, randomized, parallel, multicentre study. The primary objective was the evaluation of the effect of propionyl-L-carnitine vs placebo on maximum exercise duration using a bicycle exercise test. The primary analysis performed in the intention-to-treat population (271 and 266 patients in propionyl-L-carnitine and placebo), showed no statistically significant difference between treatments. A difference of 15 s in favour of propionyl-L-carnitine was observed in the completer/complier population (P=0.092). An a priori specified subgroup analysis on patients stratified by baseline maximum exercise duration showed a trend of improvement in propionyl-L-carnitine patients with shorter maximum exercise duration. A non a priori specified analysis in patients stratified by ejection fraction (< or = 30% vs 30-40%), showed a statistically significant difference in maximum exercise duration in favour of propionyl-L-carnitine in those patients with a higher ejection fraction (40 s, P<0.01). There were no safety issues. CONCLUSION: The study fails to meet the primary objective, but confirms the good safety profile of propionyl-L-carnitine. An exploratory non-prespecified analysis suggests that propionyl-L-carnitine improves exercise capacity in patients with preserved cardiac function. This hypothesis needs to be confirmed by a specific tailored study.  (+info)

Pharmacokinetic analysis of the cardioprotective effect of 3-(2,2, 2-trimethylhydrazinium) propionate in mice: inhibition of carnitine transport in kidney. (5/1186)

The site of action of 3-(2,2,2-trimethylhydrazinium) propionate (THP), a new cardioprotective agent, was investigated in mice and rats. I.p. administration of THP decreased the concentrations of free carnitine and long-chain acylcarnitine in heart tissue. In isolated myocytes, THP inhibited free carnitine transport with a Ki of 1340 microM, which is considerably higher than the observed serum concentration of THP. The major cause of the decreased free carnitine concentration in heart was found to be the decreased serum concentration of free carnitine that resulted from the increased renal clearance of carnitine by THP. The estimated Ki of THP for inhibiting the reabsorption of free carnitine in kidneys was 52.2 microM, which is consistent with the serum THP concentration range. No inhibition of THP on the carnitine palmitoyltransferase activity in isolated mitochondrial fractions was observed. These results indicate that the principal site of action of THP as a cardioprotective agent is the carnitine transport carrier in the kidney, but not the carrier in the heart.  (+info)

Decreased tissue distribution of L-carnitine in juvenile visceral steatosis mice. (6/1186)

We kinetically analyzed the disposition of L-carnitine of juvenile visceral steatosis (JVS) mice compared with that of normal mice to elucidate the mechanism of the systemic L-carnitine deficiency of JVS mice. There were significant differences in the plasma concentration-time course of total radioactive carnitine (L-[3H]carnitine, [acetyl-3H]carnitine, and other [acyl-3H]carnitines) between normal and JVS mice after a single i.v. or p.o. administration of L-[3H]carnitine (250 ng/kg). The oral bioavailability of L-[3H]carnitine in JVS mice (0.341) was about half of that in normal mice (0.675). The cumulative urinary excretion of total radioactive carnitine in JVS mice was about 10-fold more than that in normal mice, and the total clearance of unchanged L-[3H]carnitine for JVS mice (6.70 ml/min) was significantly higher than that for normal mice (2.45 ml/min). The distribution volume at the steady state of unchanged L-[3H]carnitine in JVS mice (1.10 liters/kg) was significantly smaller than that in normal mice (8.16 liters/kg). At 4 h after an i.v. administration, the apparent tissue-to-plasma concentration ratios of unchanged L-[3H]carnitine for various tissues of JVS mice, except for brain, were about one half to one 20th of those in normal mice. In conclusion, this in vivo disposition kinetic study of L-carnitine supports the previous in vitro finding that the L-carnitine transporter is absent or functionally deficient in JVS mice because the renal reabsorption, the intestinal absorption, and the apparent tissue-to-plasma concentration ratios in JVS mice are significantly lower than those in normal mice. The JVS mouse should be a useful experimental model for studying carnitine deficiency diseases.  (+info)

Comparisons of flux control exerted by mitochondrial outer-membrane carnitine palmitoyltransferase over ketogenesis in hepatocytes and mitochondria isolated from suckling or adult rats. (7/1186)

The primary aim of this paper was to calculate and report flux control coefficients for mitochondrial outer-membrane carnitine palmitoyltransferase (CPT I) over hepatic ketogenesis because its role in controlling this pathway during the neonatal period is of academic importance and immediate clinical relevance. Using hepatocytes isolated from suckling rats as our model system, we measured CPT I activity and carbon flux from palmitate to ketone bodies and to CO2 in the absence and presence of a range of concentrations of etomoxir. (This is converted in situ to etomoxir-CoA which is a specific inhibitor of the enzyme.) From these data we calculated the individual flux control coefficients for CPT I over ketogenesis, CO2 production and total carbon flux (0.51 +/- 0.03; -1.30 +/- 0.26; 0.55 +/- 0.07, respectively) and compared them with equivalent coefficients calculated by similar analyses [Drynan, L., Quant, P.A. & Zammit, V.A. (1996) Biochem. J. 317, 791-795] in hepatocytes isolated from adult rats (0.85 +/- 0.20; 0.23 +/- 0.06; 1.06 +/- 0.29). CPT I exerts significantly less control over ketogenesis in hepatocytes isolated from suckling rats than those from adult rats. In the suckling systems the flux control coefficients for CPT I over ketogenesis specifically and over total carbon flux (< 0.6) are not consistent with the enzyme being rate-limiting. Broadly similar results were obtained and conclusions drawn by reanalysis of previous data {from experiments in mitochondria isolated from suckling or adult rats [Krauss, S., Lascelles, C.V., Zammit, V.A. & Quant, P.A. (1996) Biochem. J. 319, 427-433]} using a different approach of control analysis, although it is not strictly valid to compare flux control coefficients from different systems. Our overall conclusion is that flux control coefficients for CPT I over oxidative fluxes from palmitate (or palmitoyl-CoA) differ markedly according to (a) the metabolic state, (b) the stage of development, (c) the specific pathway studied and (d) the model system.  (+info)

Replenishment and depletion of citric acid cycle intermediates in skeletal muscle. Indication of pyruvate carboxylation. (8/1186)

The effects of various substrates on the concentrations of free amino acids, citric acid cycle intermediates and acylcarnitines were studies in perfused hindquarter of rat in presence of glucose and insulin in order to assess regulatory mechanisms of the level of citric acid cycle intermediates in skeletal muscle. 1. Acetate and acetoacetate effected a significant increase in the level of citrate cycle intermediates and accumulation of acetylcarnitine. These changes were accompanied by a reduction in the level of alanine. The concentration of AMP was significantly elevated. 2. Muscle mitochondria fixed 14CO2 in the presence of pyruvate. The products were identified as malate or citrate when whole and disintegrated mitochondria were used respectively. The fixation was greatly stimulated by acetylcarnitine. 3. Acetylcarnitine inhibited the production of pyruvate from malate by muscle mitochondria. 4. Perfusion with 2-oxoisocaproate and 2-oxoisovalerate promoted increases in the level of citric cycle intermediates, a drop in both alanine and glutamate, and accumulation of branched-chain acylcarnitines. 2-Oxoisocaproate also caused a reduction of alanine released from the muscle. 5. Perfusion with leucine and valine did not change the concentration of citric acid cycle intermediates, but elevated glutamate and still more the concentration of alanine. 6. It is concluded that citric cycle intermediate level in the perfused resting muscle is modified by a) conditions which change the concentration of acetyl-CoA and thereby modify the rate of pyruvate carboxylation and decarboxylation of malate via malic enzyme b) conditions which change the concentration of pyruvate cause changes in alanine and cycle intermediates in the same direction via transamination reactions c) conditions which change the concentrations of 2-oxoacids which are converted to cycle intermediates via oxidation.  (+info)

Carnitine is a naturally occurring substance in the body that plays a crucial role in energy production. It transports long-chain fatty acids into the mitochondria, where they can be broken down to produce energy. Carnitine is also available as a dietary supplement and is often used to treat or prevent carnitine deficiency.

The medical definition of Carnitine is:

"A quaternary ammonium compound that occurs naturally in animal tissues, especially in muscle, heart, brain, and liver. It is essential for the transport of long-chain fatty acids into the mitochondria, where they can be oxidized to produce energy. Carnitine also functions as an antioxidant and has been studied as a potential treatment for various conditions, including heart disease, diabetes, and kidney disease."

Carnitine is also known as L-carnitine or levocarnitine. It can be found in foods such as red meat, dairy products, fish, poultry, and tempeh. In the body, carnitine is synthesized from the amino acids lysine and methionine with the help of vitamin C and iron. Some people may have a deficiency in carnitine due to genetic factors, malnutrition, or certain medical conditions, such as kidney disease or liver disease. In these cases, supplementation may be necessary to prevent or treat symptoms of carnitine deficiency.

Carnitine O-palmitoyltransferase (CPT) is an enzyme that plays a crucial role in the transport of long-chain fatty acids into the mitochondrial matrix, where they undergo beta-oxidation to produce energy. There are two main forms of this enzyme: CPT1 and CPT2.

CPT1 is located on the outer mitochondrial membrane and catalyzes the transfer of a long-chain fatty acyl group from coenzyme A (CoA) to carnitine, forming acylcarnitine. This reaction is reversible and allows for the regulation of fatty acid oxidation in response to changes in energy demand.

CPT2 is located on the inner mitochondrial membrane and catalyzes the reverse reaction, transferring the long-chain fatty acyl group from carnitine back to CoA, allowing for the entry of the fatty acid into the beta-oxidation pathway.

Deficiencies in CPT1 or CPT2 can lead to serious metabolic disorders, such as carnitine deficiency and mitochondrial myopathies, which can cause muscle weakness, cardiomyopathy, and other symptoms. Treatment may involve dietary modifications, supplementation with carnitine or medium-chain fatty acids, and in some cases, enzyme replacement therapy.

Carnitine O-acetyltransferase (COAT) is an enzyme that plays a crucial role in the transport and metabolism of fatty acids within cells. It is also known as carnitine palmitoyltransferase I (CPT I).

The primary function of COAT is to catalyze the transfer of an acetyl group from acetyl-CoA to carnitine, forming acetylcarnitine and free CoA. This reaction is essential for the entry of long-chain fatty acids into the mitochondrial matrix, where they undergo beta-oxidation to produce energy in the form of ATP.

COAT is located on the outer membrane of the mitochondria and functions as a rate-limiting enzyme in fatty acid oxidation. Its activity can be inhibited by malonyl-CoA, which is an intermediate in fatty acid synthesis. This inhibition helps regulate the balance between fatty acid oxidation and synthesis, ensuring that cells have enough energy while preventing excessive accumulation of lipids.

Deficiencies or mutations in COAT can lead to various metabolic disorders, such as carnitine palmitoyltransferase I deficiency (CPT I deficiency), which may cause symptoms like muscle weakness, hypoglycemia, and cardiomyopathy. Proper diagnosis and management of these conditions often involve dietary modifications, supplementation with carnitine, and avoidance of fasting to prevent metabolic crises.

Carnitine acyltransferases are a group of enzymes that play a crucial role in the transport and metabolism of fatty acids within cells. These enzymes are responsible for transferring acyl groups from acyl-CoAs to carnitine, forming acylcarnitines, which can then be transported across the mitochondrial membrane and into the mitochondrial matrix.

Once inside the matrix, the acyl groups can be released from carnitine and oxidized in the beta-oxidation pathway to produce energy in the form of ATP. There are three main types of carnitine acyltransferases: Carnitine palmitoyltransferase I (CPT I), located on the outer mitochondrial membrane, which activates long-chain fatty acids for transport into the mitochondria; Carnitine palmitoyltransferase II (CPT II), located on the inner mitochondrial membrane, which reconverts acylcarnitines back to acyl-CoAs for oxidation; and carnitine octanoyltransferase (CRAT), which is involved in the metabolism of medium-chain fatty acids.

Deficiencies in these enzymes can lead to various metabolic disorders, such as CPT II deficiency, which can cause muscle weakness, hypoglycemia, and cardiomyopathy. Proper regulation of carnitine acyltransferases is essential for maintaining healthy fatty acid metabolism and overall cellular function.

Acetyl-L-carnitine, also known as ALCAR, is a form of the amino acid carnitine. It is a naturally occurring substance in the body that plays a crucial role in energy production in cells, particularly within mitochondria, the "powerhouses" of the cell.

Acetyl-L-carnitine is involved in the transport of fatty acids into the mitochondria, where they can be broken down to produce energy. It also functions as an antioxidant, helping to protect cells from damage caused by free radicals.

This compound has been studied for its potential benefits in various medical conditions, including neurological disorders, cardiovascular diseases, and liver diseases. Some research suggests that Acetyl-L-carnitine may help improve cognitive function, reduce fatigue, and alleviate pain. However, more studies are needed to confirm these findings and establish the optimal dosage and safety profiles for different medical conditions.

It is important to note that while Acetyl-L-carnitine is available as a dietary supplement, its use should be discussed with a healthcare provider before starting any new supplement regimen, especially if you have a medical condition or are taking medication.

Gamma-Butyrobetaine Dioxygenase (GBD, or also known as ETHE1) is an enzyme that catalyzes the conversion of gamma-butyrobetaine to succinate and hydrogen peroxide in the final step of L-carnitine biosynthesis. This mitochondrial matrix enzyme requires iron (Fe2+) as a cofactor for its activity. Deficiency or mutations in this enzyme can lead to a rare genetic disorder called "ethylmalonic aciduria and combined oxidative phosphorylation deficiency type 6" (ETHE1), which is characterized by the accumulation of ethylmalonic acid, gamma-butyrobetaine, and other organic acids in body fluids.

Malonyl Coenzyme A (CoA) is not a medical term per se, but rather a biochemical concept. Here's the scientific or biochemical definition:

Malonyl Coenzyme A is an important intermediate in various metabolic pathways, particularly in fatty acid synthesis. It is formed through the reaction between malonic acid and coenzyme A, catalyzed by the enzyme acetyl-CoA carboxylase. Malonyl CoA plays a crucial role in the elongation step of fatty acid synthesis, where it provides the two-carbon unit that is added to a growing fatty acid chain.

In a medical context, understanding the function and regulation of Malonyl CoA metabolism can be relevant for several pathological conditions, including metabolic disorders like diabetes and obesity.

Organic cation transport proteins (OCTs) are a group of membrane transporters that facilitate the movement of organic cations across biological membranes. These transporters play an essential role in the absorption, distribution, and elimination of various endogenous and exogenous substances, including drugs and toxins.

There are four main types of OCTs, namely OCT1, OCT2, OCT3, and OCTN1 (also known as novel organic cation transporter 1 or OCT6). These proteins belong to the solute carrier (SLC) family, specifically SLC22A.

OCTs have a broad substrate specificity and can transport various organic cations, such as neurotransmitters (e.g., serotonin, dopamine, histamine), endogenous compounds (e.g., creatinine, choline), and drugs (e.g., metformin, quinidine, morphine). The transport process is typically sodium-independent and can occur in both directions, depending on the concentration gradient of the substrate.

OCTs are widely expressed in various tissues, including the liver, kidney, intestine, brain, heart, and placenta. Their expression patterns and functions vary among different OCT types, contributing to their diverse roles in physiology and pharmacology. Dysfunction of OCTs has been implicated in several diseases, such as drug toxicity, neurodegenerative disorders, and cancer.

In summary, organic cation transport proteins are membrane transporters that facilitate the movement of organic cations across biological membranes, playing crucial roles in the absorption, distribution, and elimination of various substances, including drugs and toxins.

Palmitoylcarnitine is a type of acylcarnitine, which is an ester formed from carnitine and a fatty acid. Specifically, palmitoylcarnitine consists of the long-chain fatty acid palmitate (a 16-carbon saturated fatty acid) linked to carnitine through an ester bond.

In the human body, palmitoylcarnitine plays a crucial role in the transport and metabolism of long-chain fatty acids within mitochondria, the energy-producing organelles found in cells. The process involves converting palmitate into palmitoylcarnitine by an enzyme called carnitine palmitoyltransferase I (CPT-I) in the outer mitochondrial membrane. Palmitoylcarnitine is then transported across the inner mitochondrial membrane via a specific transporter, where it is converted back to palmitate by another enzyme called carnitine palmitoyltransferase II (CPT-II). The palmitate can then undergo beta-oxidation, a process that generates energy in the form of ATP.

Abnormal levels of palmitoylcarnitine in blood or other bodily fluids may indicate an underlying metabolic disorder, such as defects in fatty acid oxidation or carnitine transport. These conditions can lead to various symptoms, including muscle weakness, cardiomyopathy, and developmental delays.

Palmitoyl Coenzyme A, often abbreviated as Palmitoyl-CoA, is a type of fatty acyl coenzyme A that plays a crucial role in the body's metabolism. It is formed from the esterification of palmitic acid (a saturated fatty acid) with coenzyme A.

Medical Definition: Palmitoyl Coenzyme A is a fatty acyl coenzyme A ester, where palmitic acid is linked to coenzyme A via an ester bond. It serves as an important intermediate in lipid metabolism and energy production, particularly through the process of beta-oxidation in the mitochondria. Palmitoyl CoA also plays a role in protein modification, known as S-palmitoylation, which can affect protein localization, stability, and function.

Acyltransferases are a group of enzymes that catalyze the transfer of an acyl group (a functional group consisting of a carbon atom double-bonded to an oxygen atom and single-bonded to a hydrogen atom) from one molecule to another. This transfer involves the formation of an ester bond between the acyl group donor and the acyl group acceptor.

Acyltransferases play important roles in various biological processes, including the biosynthesis of lipids, fatty acids, and other metabolites. They are also involved in the detoxification of xenobiotics (foreign substances) by catalyzing the addition of an acyl group to these compounds, making them more water-soluble and easier to excrete from the body.

Examples of acyltransferases include serine palmitoyltransferase, which is involved in the biosynthesis of sphingolipids, and cholesteryl ester transfer protein (CETP), which facilitates the transfer of cholesteryl esters between lipoproteins.

Acyltransferases are classified based on the type of acyl group they transfer and the nature of the acyl group donor and acceptor molecules. They can be further categorized into subclasses based on their sequence similarities, three-dimensional structures, and evolutionary relationships.

Betaine, also known as trimethylglycine, is a naturally occurring compound that can be found in various foods such as beets, spinach, and whole grains. In the body, betaine functions as an osmolyte, helping to regulate water balance in cells, and as a methyl donor, contributing to various metabolic processes including the conversion of homocysteine to methionine.

In medical terms, betaine is also used as a dietary supplement and medication. Betaine hydrochloride is a form of betaine that is sometimes used as a supplement to help with digestion by providing additional stomach acid. Betaine anhydrous, on the other hand, is often used as a supplement for improving athletic performance and promoting liver health.

Betaine has also been studied for its potential role in protecting against various diseases, including cardiovascular disease, diabetes, and neurological disorders. However, more research is needed to fully understand its mechanisms of action and therapeutic potential.

Acyl Coenzyme A (often abbreviated as Acetyl-CoA or Acyl-CoA) is a crucial molecule in metabolism, particularly in the breakdown and oxidation of fats and carbohydrates to produce energy. It is a thioester compound that consists of a fatty acid or an acetate group linked to coenzyme A through a sulfur atom.

Acyl CoA plays a central role in several metabolic pathways, including:

1. The citric acid cycle (Krebs cycle): In the mitochondria, Acyl-CoA is formed from the oxidation of fatty acids or the breakdown of certain amino acids. This Acyl-CoA then enters the citric acid cycle to produce high-energy electrons, which are used in the electron transport chain to generate ATP (adenosine triphosphate), the main energy currency of the cell.
2. Beta-oxidation: The breakdown of fatty acids occurs in the mitochondria through a process called beta-oxidation, where Acyl-CoA is sequentially broken down into smaller units, releasing acetyl-CoA, which then enters the citric acid cycle.
3. Ketogenesis: In times of low carbohydrate availability or during prolonged fasting, the liver can produce ketone bodies from acetyl-CoA to supply energy to other organs, such as the brain and heart.
4. Protein synthesis: Acyl-CoA is also involved in the modification of proteins by attaching fatty acid chains to them (a process called acetylation), which can influence protein function and stability.

In summary, Acyl Coenzyme A is a vital molecule in metabolism that connects various pathways related to energy production, fatty acid breakdown, and protein modification.

I am sorry for any confusion, but "Pentanoic Acids" is not a recognized medical term or concept. Pentanoic acid, also known as valeric acid, is an organic compound with the formula CH3CH2CH2CH2CO2H. It is a fatty acid with five carbon atoms, and it may have some uses in industry, but it does not have specific relevance to medical definition or healthcare.

Coenzyme A, often abbreviated as CoA or sometimes holo-CoA, is a coenzyme that plays a crucial role in several important chemical reactions in the body, particularly in the metabolism of carbohydrates, fatty acids, and amino acids. It is composed of a pantothenic acid (vitamin B5) derivative called pantothenate, an adenosine diphosphate (ADP) molecule, and a terminal phosphate group.

Coenzyme A functions as a carrier molecule for acetyl groups, which are formed during the breakdown of carbohydrates, fatty acids, and some amino acids. The acetyl group is attached to the sulfur atom in CoA, forming acetyl-CoA, which can then be used as a building block for various biochemical pathways, such as the citric acid cycle (Krebs cycle) and fatty acid synthesis.

In summary, Coenzyme A is a vital coenzyme that helps facilitate essential metabolic processes by carrying and transferring acetyl groups in the body.

I'd be happy to help! However, I believe there might be a slight misunderstanding in your question. Mitochondria are organelles found in the cells of all complex living organisms, including humans, while the liver is a large, solid organ located in the upper right portion of the abdomen. They don't have a medical definition together. I can certainly provide you with separate definitions for each:

1. Mitochondria: These are double-membrane-bound cellular organelles that generate most of the chemical energy needed to power the cell's biochemical reactions. Commonly known as the "powerhouse of the cell," mitochondria convert organic substrates, such as glucose, fatty acids, and amino acids, into adenosine triphosphate (ATP) through a process called oxidative phosphorylation. Mitochondria are dynamic structures that can change their shape, size, and number through fission (division) and fusion (merging) processes. They play essential roles in various cellular functions, including calcium signaling, apoptosis (programmed cell death), and the regulation of cellular metabolism.

2. Liver: The liver is a large, lobulated organ that lies mainly in the upper right portion of the abdominal cavity, just below the diaphragm. It plays a crucial role in various physiological functions, such as detoxification, protein synthesis, metabolism, and nutrient storage. The liver is responsible for removing toxins from the bloodstream, producing bile to aid in digestion, regulating glucose levels, synthesizing plasma proteins, and storing glycogen, vitamins, and minerals. It also contributes to the metabolism of carbohydrates, lipids, and amino acids, helping maintain energy homeostasis in the body.

I hope this clarifies any confusion! If you have any further questions or need more information, please don't hesitate to ask.

Vitamin B deficiency refers to a condition where an individual's body lacks adequate amounts of one or more essential Vitamin B compounds, including Vitamin B1 (thiamin), Vitamin B2 (riboflavin), Vitamin B3 (niacin), Vitamin B5 (pantothenic acid), Vitamin B6 (pyridoxine), Vitamin B7 (biotin), Vitamin B9 (folate), and Vitamin B12 (cobalamin). These water-soluble vitamins play crucial roles in various bodily functions, such as energy production, nerve function, DNA repair, and the formation of red blood cells.

Deficiency in any of these Vitamin B compounds can lead to specific health issues. For instance:

1. Vitamin B1 (thiamin) deficiency can cause beriberi, a condition characterized by muscle weakness, peripheral neuropathy, and heart failure.
2. Vitamin B2 (riboflavin) deficiency may result in ariboflavinosis, which presents with inflammation of the mouth and tongue, anemia, and skin disorders.
3. Vitamin B3 (niacin) deficiency can lead to pellagra, marked by diarrhea, dermatitis, dementia, and, if left untreated, death.
4. Vitamin B5 (pantothenic acid) deficiency is rare but can cause acne-like skin lesions and neurological symptoms.
5. Vitamin B6 (pyridoxine) deficiency may result in anemia, peripheral neuropathy, seizures, and skin disorders.
6. Vitamin B7 (biotin) deficiency can cause hair loss, skin rashes, and neurological symptoms.
7. Vitamin B9 (folate) deficiency can lead to megaloblastic anemia, neural tube defects in fetuses during pregnancy, and increased homocysteine levels, which may contribute to cardiovascular disease.
8. Vitamin B12 (cobalamin) deficiency can cause pernicious anemia, characterized by fatigue, weakness, neurological symptoms, and, if left untreated, irreversible nerve damage.

Deficiencies in these vitamins can arise from inadequate dietary intake, malabsorption syndromes, or certain medications that interfere with absorption or metabolism. It is essential to maintain a balanced diet and consider supplementation if necessary under the guidance of a healthcare professional.

Epoxy compounds, also known as epoxy resins, are a type of thermosetting polymer characterized by the presence of epoxide groups in their molecular structure. An epoxide group is a chemical functional group consisting of an oxygen atom double-bonded to a carbon atom, which is itself bonded to another carbon atom.

Epoxy compounds are typically produced by reacting a mixture of epichlorohydrin and bisphenol-A or other similar chemicals under specific conditions. The resulting product is a two-part system consisting of a resin and a hardener, which must be mixed together before use.

Once the two parts are combined, a chemical reaction takes place that causes the mixture to cure or harden into a solid material. This curing process can be accelerated by heat, and once fully cured, epoxy compounds form a strong, durable, and chemically resistant material that is widely used in various industrial and commercial applications.

In the medical field, epoxy compounds are sometimes used as dental restorative materials or as adhesives for bonding medical devices or prosthetics. However, it's important to note that some people may have allergic reactions to certain components of epoxy compounds, so their use must be carefully evaluated and monitored in a medical context.

Fatty acids are carboxylic acids with a long aliphatic chain, which are important components of lipids and are widely distributed in living organisms. They can be classified based on the length of their carbon chain, saturation level (presence or absence of double bonds), and other structural features.

The two main types of fatty acids are:

1. Saturated fatty acids: These have no double bonds in their carbon chain and are typically solid at room temperature. Examples include palmitic acid (C16:0) and stearic acid (C18:0).
2. Unsaturated fatty acids: These contain one or more double bonds in their carbon chain and can be further classified into monounsaturated (one double bond) and polyunsaturated (two or more double bonds) fatty acids. Examples of unsaturated fatty acids include oleic acid (C18:1, monounsaturated), linoleic acid (C18:2, polyunsaturated), and alpha-linolenic acid (C18:3, polyunsaturated).

Fatty acids play crucial roles in various biological processes, such as energy storage, membrane structure, and cell signaling. Some essential fatty acids cannot be synthesized by the human body and must be obtained through dietary sources.

Ketone bodies, also known as ketones or ketoacids, are organic compounds that are produced by the liver during the metabolism of fats when carbohydrate intake is low. They include acetoacetate (AcAc), beta-hydroxybutyrate (BHB), and acetone. These molecules serve as an alternative energy source for the body, particularly for the brain and heart, when glucose levels are insufficient to meet energy demands.

In a healthy individual, ketone bodies are present in low concentrations; however, during periods of fasting, starvation, or intense physical exertion, ketone production increases significantly. In some pathological conditions like uncontrolled diabetes mellitus, the body may produce excessive amounts of ketones, leading to a dangerous metabolic state called diabetic ketoacidosis (DKA).

Elevated levels of ketone bodies can be detected in blood or urine and are often used as an indicator of metabolic status. Monitoring ketone levels is essential for managing certain medical conditions, such as diabetes, where maintaining optimal ketone concentrations is crucial to prevent complications.

The liver is a large, solid organ located in the upper right portion of the abdomen, beneath the diaphragm and above the stomach. It plays a vital role in several bodily functions, including:

1. Metabolism: The liver helps to metabolize carbohydrates, fats, and proteins from the food we eat into energy and nutrients that our bodies can use.
2. Detoxification: The liver detoxifies harmful substances in the body by breaking them down into less toxic forms or excreting them through bile.
3. Synthesis: The liver synthesizes important proteins, such as albumin and clotting factors, that are necessary for proper bodily function.
4. Storage: The liver stores glucose, vitamins, and minerals that can be released when the body needs them.
5. Bile production: The liver produces bile, a digestive juice that helps to break down fats in the small intestine.
6. Immune function: The liver plays a role in the immune system by filtering out bacteria and other harmful substances from the blood.

Overall, the liver is an essential organ that plays a critical role in maintaining overall health and well-being.

Ergothioneine is a naturally occurring antioxidant compound that is found in various foods such as mushrooms, some types of beans, and certain grains. It is also produced by some bacteria that live in the human body. Ergothioneine has been shown to have potential health benefits due to its antioxidant properties, which help protect cells from damage caused by free radicals. Some studies suggest that ergothioneine may have neuroprotective effects and could play a role in preventing neurodegenerative diseases such as Parkinson's and Alzheimer's. However, more research is needed to fully understand the potential health benefits of this compound.

Starvation is a severe form of malnutrition, characterized by insufficient intake of calories and nutrients to meet the body's energy requirements. This leads to a catabolic state where the body begins to break down its own tissues for energy, resulting in significant weight loss, muscle wasting, and weakness. Prolonged starvation can also lead to serious medical complications such as organ failure, electrolyte imbalances, and even death. It is typically caused by a lack of access to food due to poverty, famine, or other social or economic factors, but can also be a result of severe eating disorders such as anorexia nervosa.

3-Hydroxybutyric acid, also known as β-hydroxybutyric acid, is a type of ketone body that is produced in the liver during the metabolism of fatty acids. It is a colorless, slightly water-soluble compound with a bitter taste and an unpleasant odor.

In the body, 3-hydroxybutyric acid is produced when there is not enough glucose available to meet the body's energy needs, such as during fasting, starvation, or prolonged intense exercise. It can also be produced in large amounts in people with uncontrolled diabetes, particularly during a condition called diabetic ketoacidosis.

3-Hydroxybutyric acid is an important source of energy for the brain and other organs during periods of low glucose availability. However, high levels of 3-hydroxybutyric acid in the blood can lead to a condition called ketosis, which can cause symptoms such as nausea, vomiting, abdominal pain, and confusion. If left untreated, ketosis can progress to diabetic ketoacidosis, a potentially life-threatening complication of diabetes.

Inborn errors of lipid metabolism refer to genetic disorders that affect the body's ability to break down and process lipids (fats) properly. These disorders are caused by defects in genes that code for enzymes or proteins involved in lipid metabolism. As a result, toxic levels of lipids or their intermediates may accumulate in the body, leading to various health issues, which can include neurological problems, liver dysfunction, muscle weakness, and cardiovascular disease.

There are several types of inborn errors of lipid metabolism, including:

1. Disorders of fatty acid oxidation: These disorders affect the body's ability to convert long-chain fatty acids into energy, leading to muscle weakness, hypoglycemia, and cardiomyopathy. Examples include medium-chain acyl-CoA dehydrogenase deficiency (MCAD) and very long-chain acyl-CoA dehydrogenase deficiency (VLCAD).
2. Disorders of cholesterol metabolism: These disorders affect the body's ability to process cholesterol, leading to an accumulation of cholesterol or its intermediates in various tissues. Examples include Smith-Lemli-Opitz syndrome and lathosterolosis.
3. Disorders of sphingolipid metabolism: These disorders affect the body's ability to break down sphingolipids, leading to an accumulation of these lipids in various tissues. Examples include Gaucher disease, Niemann-Pick disease, and Fabry disease.
4. Disorders of glycerophospholipid metabolism: These disorders affect the body's ability to break down glycerophospholipids, leading to an accumulation of these lipids in various tissues. Examples include rhizomelic chondrodysplasia punctata and abetalipoproteinemia.

Inborn errors of lipid metabolism are typically diagnosed through genetic testing and biochemical tests that measure the activity of specific enzymes or the levels of specific lipids in the body. Treatment may include dietary modifications, supplements, enzyme replacement therapy, or gene therapy, depending on the specific disorder and its severity.

Reye Syndrome is a rare but serious condition that primarily affects children and teenagers, particularly those who have recently recovered from viral infections such as chickenpox or flu. It is characterized by rapidly progressive encephalopathy (brain dysfunction) and fatty degeneration of the liver.

The exact cause of Reye Syndrome remains unknown, but it has been linked to the use of aspirin and other salicylate-containing medications during viral illnesses. The American Academy of Pediatrics recommends avoiding the use of aspirin in children and teenagers with chickenpox or flu-like symptoms due to this association.

Early symptoms of Reye Syndrome include persistent vomiting, diarrhea, and listlessness. As the condition progresses, symptoms can worsen and may include disorientation, seizures, coma, and even death in severe cases. Diagnosis is typically based on clinical presentation, laboratory tests, and sometimes a liver biopsy.

Treatment for Reye Syndrome involves supportive care, such as fluid and electrolyte management, addressing metabolic abnormalities, controlling intracranial pressure, and providing ventilatory support if necessary. Early recognition and intervention are crucial to improving outcomes in affected individuals.

Oxidation-Reduction (redox) reactions are a type of chemical reaction involving a transfer of electrons between two species. The substance that loses electrons in the reaction is oxidized, and the substance that gains electrons is reduced. Oxidation and reduction always occur together in a redox reaction, hence the term "oxidation-reduction."

In biological systems, redox reactions play a crucial role in many cellular processes, including energy production, metabolism, and signaling. The transfer of electrons in these reactions is often facilitated by specialized molecules called electron carriers, such as nicotinamide adenine dinucleotide (NAD+/NADH) and flavin adenine dinucleotide (FAD/FADH2).

The oxidation state of an element in a compound is a measure of the number of electrons that have been gained or lost relative to its neutral state. In redox reactions, the oxidation state of one or more elements changes as they gain or lose electrons. The substance that is oxidized has a higher oxidation state, while the substance that is reduced has a lower oxidation state.

Overall, oxidation-reduction reactions are fundamental to the functioning of living organisms and are involved in many important biological processes.

Palmitic acid is a type of saturated fatty acid, which is a common component in many foods and also produced by the body. Its chemical formula is C16:0, indicating that it contains 16 carbon atoms and no double bonds. Palmitic acid is found in high concentrations in animal fats, such as butter, lard, and beef tallow, as well as in some vegetable oils, like palm kernel oil and coconut oil.

In the human body, palmitic acid can be synthesized from other substances or absorbed through the diet. It plays a crucial role in various biological processes, including energy storage, membrane structure formation, and signaling pathways regulation. However, high intake of palmitic acid has been linked to an increased risk of developing cardiovascular diseases due to its potential to raise low-density lipoprotein (LDL) cholesterol levels in the blood.

It is essential to maintain a balanced diet and consume palmitic acid-rich foods in moderation, along with regular exercise and a healthy lifestyle, to reduce the risk of chronic diseases.

"Inbred strains of rats" are genetically identical rodents that have been produced through many generations of brother-sister mating. This results in a high degree of homozygosity, where the genes at any particular locus in the genome are identical in all members of the strain.

Inbred strains of rats are widely used in biomedical research because they provide a consistent and reproducible genetic background for studying various biological phenomena, including the effects of drugs, environmental factors, and genetic mutations on health and disease. Additionally, inbred strains can be used to create genetically modified models of human diseases by introducing specific mutations into their genomes.

Some commonly used inbred strains of rats include the Wistar Kyoto (WKY), Sprague-Dawley (SD), and Fischer 344 (F344) rat strains. Each strain has its own unique genetic characteristics, making them suitable for different types of research.

"Palmitates" are salts or esters of palmitic acid, a saturated fatty acid that is commonly found in animals and plants. Palmitates can be found in various substances, including cosmetics, food additives, and medications. For example, sodium palmitate is a common ingredient in soaps and detergents, while retinyl palmitate is a form of vitamin A used in skin care products and dietary supplements.

In a medical context, "palmitates" may be mentioned in the results of laboratory tests that measure lipid metabolism or in discussions of nutrition and dietary fats. However, it is important to note that "palmitates" themselves are not typically a focus of medical diagnosis or treatment, but rather serve as components of various substances that may have medical relevance.

Microbodies are small, membrane-bound organelles found in the cells of eukaryotic organisms. They typically measure between 0.2 to 0.5 micrometers in diameter and play a crucial role in various metabolic processes, particularly in the detoxification of harmful substances and the synthesis of lipids.

There are several types of microbodies, including:

1. Peroxisomes: These are the most common type of microbody. They contain enzymes that help break down fatty acids and amino acids, producing hydrogen peroxide as a byproduct. Another set of enzymes within peroxisomes then converts the harmful hydrogen peroxide into water and oxygen, thus detoxifying the cell.
2. Glyoxysomes: These microbodies are primarily found in plants and some fungi. They contain enzymes involved in the glyoxylate cycle, a metabolic pathway that helps convert stored fats into carbohydrates during germination.
3. Microbody-like particles (MLPs): These are smaller organelles found in certain protists and algae. Their functions are not well understood but are believed to be involved in lipid metabolism.

It is important to note that microbodies do not have a uniform structure or function across all eukaryotic cells, and their specific roles can vary depending on the organism and cell type.

Hyperammonemia is a medical condition characterized by an excessively high level of ammonia (a toxic byproduct of protein metabolism) in the blood. This can lead to serious neurological symptoms and complications, as ammonia is highly toxic to the brain. Hyperammonemia can be caused by various underlying conditions, including liver disease, genetic disorders that affect ammonia metabolism, certain medications, and infections. It is important to diagnose and treat hyperammonemia promptly to prevent long-term neurological damage or even death. Treatment typically involves addressing the underlying cause of the condition, as well as providing supportive care such as administering medications that help remove ammonia from the blood.

Methylhydrazines are a class of organic compounds that contain a hydrazine functional group with one or more methyl substituents. Hydrazine is a simple inorganic compound with the formula N2H4, and it consists of a nitrogen atom bonded to four hydrogen atoms through nitrogen-hydrogen covalent bonds. When one or more of these hydrogens are replaced by a methyl group (CH3), we get methylhydrazines.

The most common methylhydrazine is monomethylhydrazine (MMH), which has the molecular formula CH6N2. It is an colorless, oily liquid with an ammonia-like odor and is highly toxic and reactive. MMH is used as a rocket propellant due to its high specific impulse and density.

Another example of methylhydrazine is unsymmetrical dimethylhydrazine (UDMH), which has the molecular formula C2H8N2. UDMH is also a colorless, oily liquid with an ammonia-like odor and is used as a rocket propellant.

It's important to note that methylhydrazines are highly toxic and reactive compounds, and they require careful handling and storage. They can cause harm to the skin, eyes, respiratory system, and nervous system, and prolonged exposure can lead to serious health effects or death.

Mersalyl is not a medical condition or diagnosis, but rather a pharmaceutical compound. It is a type of organic mercurial salt that was historically used in medicine as a diuretic and an antimicrobial agent. However, its use has been largely discontinued due to the toxic effects of mercury on the human body. Therefore, there isn't a medical definition for 'Mersalyl'.

Caprylates are the salts or esters of capric acid, a saturated fatty acid with a chain length of 8 carbon atoms. In medical and biological contexts, caprylate refers to the anion (negatively charged ion) form of capric acid, which has the chemical formula C8H17O2-. Caprylates are used in various applications, including as food additives, pharmaceuticals, and personal care products.

Some examples of caprylate compounds include:

* Sodium caprylate (sodium octanoate): a sodium salt commonly used as a preservative and flavor enhancer in foods.
* Calcium caprylate (calcium octanoate): a calcium salt used as an emulsifier in food products and as a stabilizer in cosmetics.
* Caprylic acid/caprylate triglycerides: esters of glycerin with caprylic acid, used as emollients and solvents in skin care products and pharmaceuticals.

Caprylates have antimicrobial properties against certain bacteria, fungi, and viruses, making them useful in various medical applications. For instance, sodium caprylate is sometimes used as an antifungal agent to treat conditions like candidiasis (yeast infections). However, more research is needed to fully understand the potential benefits and risks of using caprylates for medicinal purposes.

Mitochondria in muscle, also known as the "powerhouses" of the cell, are organelles that play a crucial role in generating energy for muscle cells through a process called cellular respiration. They convert the chemical energy found in glucose and oxygen into ATP (adenosine triphosphate), which is the main source of energy used by cells.

Muscle cells contain a high number of mitochondria due to their high energy demands for muscle contraction and relaxation. The number and size of mitochondria in muscle fibers can vary depending on the type of muscle fiber, with slow-twitch, aerobic fibers having more numerous and larger mitochondria than fast-twitch, anaerobic fibers.

Mitochondrial dysfunction has been linked to various muscle disorders, including mitochondrial myopathies, which are characterized by muscle weakness, exercise intolerance, and other symptoms related to impaired energy production in the muscle cells.

Hydroxybutyrates are compounds that contain a hydroxyl group (-OH) and a butyric acid group. More specifically, in the context of clinical medicine and biochemistry, β-hydroxybutyrate (BHB) is often referred to as a "ketone body."

Ketone bodies are produced by the liver during periods of low carbohydrate availability, such as during fasting, starvation, or a high-fat, low-carbohydrate diet. BHB is one of three major ketone bodies, along with acetoacetate and acetone. These molecules serve as alternative energy sources for the brain and other tissues when glucose levels are low.

In some pathological states, such as diabetic ketoacidosis, the body produces excessive amounts of ketone bodies, leading to a life-threatening metabolic acidosis. Elevated levels of BHB can also be found in other conditions like alcoholism, severe illnesses, and high-fat diets.

It is important to note that while BHB is a hydroxybutyrate, not all hydroxybutyrates are ketone bodies. The term "hydroxybutyrates" can refer to any compound containing both a hydroxyl group (-OH) and a butyric acid group.

A muscle is a soft tissue in our body that contracts to produce force and motion. It is composed mainly of specialized cells called muscle fibers, which are bound together by connective tissue. There are three types of muscles: skeletal (voluntary), smooth (involuntary), and cardiac. Skeletal muscles attach to bones and help in movement, while smooth muscles are found within the walls of organs and blood vessels, helping with functions like digestion and circulation. Cardiac muscle is the specific type that makes up the heart, allowing it to pump blood throughout the body.

Clofibrate is a medication that belongs to the class of drugs known as fibrates. It is primarily used to lower elevated levels of cholesterol and other fats (lipids) in the blood, specifically low-density lipoprotein (LDL), or "bad" cholesterol, and triglycerides, while increasing high-density lipoprotein (HDL), or "good" cholesterol. Clofibrate works by reducing the production of very-low-density lipoproteins (VLDL) in the liver, which in turn lowers triglyceride levels and indirectly reduces LDL cholesterol levels.

Clofibrate is available in oral tablet form and is typically prescribed for patients with high cholesterol or triglycerides who are at risk of cardiovascular disease, such as those with a history of heart attacks, strokes, or peripheral artery disease. It is important to note that clofibrate should be used in conjunction with lifestyle modifications, including a healthy diet, regular exercise, and smoking cessation.

Like all medications, clofibrate can have side effects, some of which may be serious. Common side effects include stomach upset, diarrhea, gas, and changes in taste. Less commonly, clofibrate can cause more severe side effects such as liver or muscle damage, gallstones, and an increased risk of developing certain types of cancer. Patients taking clofibrate should be monitored regularly by their healthcare provider to ensure that the medication is working effectively and to monitor for any potential side effects.

In the context of medicine and pharmacology, "kinetics" refers to the study of how a drug moves throughout the body, including its absorption, distribution, metabolism, and excretion (often abbreviated as ADME). This field is called "pharmacokinetics."

1. Absorption: This is the process of a drug moving from its site of administration into the bloodstream. Factors such as the route of administration (e.g., oral, intravenous, etc.), formulation, and individual physiological differences can affect absorption.

2. Distribution: Once a drug is in the bloodstream, it gets distributed throughout the body to various tissues and organs. This process is influenced by factors like blood flow, protein binding, and lipid solubility of the drug.

3. Metabolism: Drugs are often chemically modified in the body, typically in the liver, through processes known as metabolism. These changes can lead to the formation of active or inactive metabolites, which may then be further distributed, excreted, or undergo additional metabolic transformations.

4. Excretion: This is the process by which drugs and their metabolites are eliminated from the body, primarily through the kidneys (urine) and the liver (bile).

Understanding the kinetics of a drug is crucial for determining its optimal dosing regimen, potential interactions with other medications or foods, and any necessary adjustments for special populations like pediatric or geriatric patients, or those with impaired renal or hepatic function.

Acyl-CoA dehydrogenase is a group of enzymes that play a crucial role in the body's energy production process. Specifically, they are involved in the breakdown of fatty acids within the cells.

More technically, acyl-CoA dehydrogenases catalyze the removal of electrons from the thiol group of acyl-CoAs, forming a trans-double bond and generating FADH2. This reaction is the first step in each cycle of fatty acid beta-oxidation, which occurs in the mitochondria of cells.

There are several different types of acyl-CoA dehydrogenases, each specific to breaking down different lengths of fatty acids. For example, very long-chain acyl-CoA dehydrogenase (VLCAD) is responsible for breaking down longer chain fatty acids, while medium-chain acyl-CoA dehydrogenase (MCAD) breaks down medium-length chains.

Deficiencies in these enzymes can lead to various metabolic disorders, such as MCAD deficiency or LC-FAOD (long-chain fatty acid oxidation disorders), which can cause symptoms like vomiting, lethargy, and muscle weakness, especially during periods of fasting or illness.

Lipid metabolism is the process by which the body breaks down and utilizes lipids (fats) for various functions, such as energy production, cell membrane formation, and hormone synthesis. This complex process involves several enzymes and pathways that regulate the digestion, absorption, transport, storage, and consumption of fats in the body.

The main types of lipids involved in metabolism include triglycerides, cholesterol, phospholipids, and fatty acids. The breakdown of these lipids begins in the digestive system, where enzymes called lipases break down dietary fats into smaller molecules called fatty acids and glycerol. These molecules are then absorbed into the bloodstream and transported to the liver, which is the main site of lipid metabolism.

In the liver, fatty acids may be further broken down for energy production or used to synthesize new lipids. Excess fatty acids may be stored as triglycerides in specialized cells called adipocytes (fat cells) for later use. Cholesterol is also metabolized in the liver, where it may be used to synthesize bile acids, steroid hormones, and other important molecules.

Disorders of lipid metabolism can lead to a range of health problems, including obesity, diabetes, cardiovascular disease, and non-alcoholic fatty liver disease (NAFLD). These conditions may be caused by genetic factors, lifestyle habits, or a combination of both. Proper diagnosis and management of lipid metabolism disorders typically involves a combination of dietary changes, exercise, and medication.

The myocardium is the middle layer of the heart wall, composed of specialized cardiac muscle cells that are responsible for pumping blood throughout the body. It forms the thickest part of the heart wall and is divided into two sections: the left ventricle, which pumps oxygenated blood to the rest of the body, and the right ventricle, which pumps deoxygenated blood to the lungs.

The myocardium contains several types of cells, including cardiac muscle fibers, connective tissue, nerves, and blood vessels. The muscle fibers are arranged in a highly organized pattern that allows them to contract in a coordinated manner, generating the force necessary to pump blood through the heart and circulatory system.

Damage to the myocardium can occur due to various factors such as ischemia (reduced blood flow), infection, inflammation, or genetic disorders. This damage can lead to several cardiac conditions, including heart failure, arrhythmias, and cardiomyopathy.

L-Carnitine, acetyl-l-carnitine, and propionyl-l-carnitine are available in dietary supplement pills or powders, with a daily ... Carnitine exists as one of two stereoisomers (the two enantiomers d-carnitine (S-(+)-) and l-carnitine (R-(−)-)). Both are ... Two types of carnitine deficiency states exist. Primary carnitine deficiency is a genetic disorder of the cellular carnitine- ... Even carnitine-poor diets have little effect on total carnitine content, as the kidneys conserve carnitine. In general, ...
The enzyme carnitine decarboxylase (EC 4.1.1.42) catalyzes the chemical reaction carnitine ⇌ {\displaystyle \rightleftharpoons ... Khairallah EA, Wolf G (1967). "Carnitine decarboxylase. The conversion of carnitine to beta-methylcholine". J. Biol. Chem. 242 ... The systematic name of this enzyme class is carnitine carboxy-lyase (2-methylcholine-forming). This enzyme is also called ... carnitine carboxy-lyase.[citation needed] It employs one cofactor, ATP. ...
... is a method for the endogenous production of L-carnitine, a molecule that is essential for energy ... The carnitine biosynthesis pathway is highly conserved among many eukaryotes and some prokaryotes. L-Carnitine is ... Carnitine. Vitam. Horm. 1957, 15, 73-118. Bremer, J. Carnitine - metabolism and functions. Physiol. Rev. 1983, 63, 1420-1480. ... Dietary carnitine intake related to skeletal muscle and plasma carnitine concentrations in adult men and women. Am. J. Clin. ...
In enzymology, a carnitine dehydratase (EC 4.2.1.89) is an enzyme that catalyzes the chemical reaction L-carnitine ⇌ {\ ... This enzyme is also called L-carnitine hydro-lyase. Kano K, Imai K, Nishimori H (1986). "Production of L-carnitine". Chem. ... The systematic name of this enzyme class is L-carnitine hydro-lyase [4-(trimethylammonio)but-2-enoate-forming]. ... displaystyle \rightleftharpoons } 4-(trimethylammonio)but-2-enoate + H2O Hence, this enzyme has one substrate, L-carnitine, and ...
In enzymology, a carnitine 3-dehydrogenase (EC 1.1.1.108) is an enzyme that catalyzes the chemical reaction carnitine + NAD+ ... carnitine+dehydrogenase at the U.S. National Library of Medicine Medical Subject Headings (MeSH) EC 1.1.1.108 Portal: Biology v ... The systematic name of this enzyme class is carnitine:NAD+ 3-oxidoreductase. Aurich H, Kleber HP, Sorger H, Tauchert H (1968 ... the two substrates of this enzyme are carnitine and NAD+, whereas its 3 products are 3-dehydrocarnitine, NADH, and H+. This ...
... (CROT or COT) is a member of the transferase family, more specifically a carnitine ... Valkner, K. J.; Bieber, L. L. (1982-07-14). "The sidedness of carnitine acetyltransferase and carnitine octanoyltransferase of ... Other names in common use include medium-chain/long-chain carnitine acyltransferase, carnitine medium-chain acyltransferase, ... Ozasa, H.; Miyazawa, S.; Osumi, T. (August 1983). "Biosynthesis of carnitine octanoyltransferase and carnitine ...
Carnitine O-palmitoyltransferase 2, mitochondrial is an enzyme that in humans is encoded by the CPT2 gene. Carnitine ... CPT2 together with carnitine palmitoyltransferase I oxidizes long-chain fatty acids in the mitochondria. Defects in this gene ... Verderio E, Cavadini P, Montermini L, Wang H, Lamantea E, Finocchiaro G, DiDonato S, Gellera C, Taroni F (1995). "Carnitine ... van der Leij FR, Huijkman NC, Boomsma C, Kuipers JR, Bartelds B (2000). "Genomics of the human carnitine acyltransferase genes ...
... (also called carnitine palmitoyltransferase) is a mitochondrial transferase enzyme (EC 2.3. ... A related transferase is carnitine acyltransferase. Palmitoylcarnitine Palmitoyl CoA There are four different forms of CPT in ... PDOC00402 - Acyltransferases ChoActase / COT / CPT family in PROSITE Choline/Carnitine o-acyltransferase family[permanent dead ... humans: CPT1A - associated with Carnitine palmitoyltransferase I deficiency CPT1B CPT1C CPT2 - associated with carnitine ...
... (CACT) is responsible for passive transport of carnitine and carnitine-fatty acid complexes ... A disorder is associated with carnitine-acylcarnitine translocase deficiency. This disorder disrupts the carnitine shuttle ... returning one molecule of carnitine from the matrix to the intermembrane space as one molecule of fatty acyl-carnitine moves ... Fatty acyl-carnitine can diffuse from the cytosol across the porous outer mitochondrial membrane to the intermembrane space, ...
... carnitine acetyl coenzyme A transferase, carnitine acetylase, carnitine acetyltransferase, carnitine-acetyl-CoA transferase, ... Like CoA, carnitine forms a hydrogen bond with the ε2 nitrogen on His343. In the case of carnitine, the bond is formed with its ... Carnitine O-acetyltransferase also called carnitine acetyltransferase (CRAT, or CAT) (EC 2.3.1.7) is an enzyme that encoded by ... "Entrez Gene: CRAT carnitine acetyltransferase". McGarry JD, Brown NF (Feb 1997). "The mitochondrial carnitine ...
... (CPT1) also known as carnitine acyltransferase I, CPTI, CAT1, CoA:carnitine acyl transferase ( ... Carnitine palmitoyltransferase I is the first component and rate-limiting step of the carnitine palmitoyltransferase system, ... One such mechanism based upon a carnitine acetyltransferase model is shown below in which the His 473 deprotonates carnitine ... such as carnitine acetyltransferase (CRAT). An important structural difference between CPT1 and CPT2, CRAT and carnitine ...
Carnitine is an important amino acid for fatty acid metabolism. When carnitine cannot be transported into tissues, fatty acid ... Some may have carnitine deficiency secondary to another metabolic condition or due to maternal carnitine deficiency. Proper ... Systemic primary carnitine deficiency Orphanet Online Mendelian Inheritance in Man (OMIM): 212140 Carnitine transporter ... Decreased levels of plasma carnitine inhibit fatty acid oxidation during times of excessive energy demand. Carnitine is needed ...
Primary carnitine deficiency Carnitine palmitoyltransferase II deficiency Bennett, Michael J.; Santani, Avni B. (1993-01-01). " ... and elevated levels of carnitine in the blood. Mutations in the CPT1A gene cause carnitine palmitoyltransferase I deficiency by ... Carnitine palmitoyltransferase I deficiency is a rare metabolic disorder that prevents the body from converting certain fats ... It is caused by a mutation in CPT1A on chromosome 11.[citation needed] Carnitine, a natural substance acquired mostly through ...
Carnitine O-palmitoyltransferase Carnitine palmitoyltransferase I deficiency Fasciculation Myokymia Primary carnitine ... The "carnitine shuttle" is composed of three enzymes that utilize carnitine to facilitate the import of hydrophobic long-chain ... Longo N, Amat, San Filippo C, Pasquali M (2006). "Disorders of Carnitine Transport and the Carnitine Cycle". Am J Med Genet C ... The Role of Carnitine in Enhancing Physical Performance. National Academies Press (US). "Carnitine palmitoyltransferase II ...
... (GPLC) is a propionyl ester of carnitine that includes an additional glycine component. Due to ... 6 (9). Smith, WA; Fry, AC; Tschume, LC; Bloomer, RJ (Feb 2008). "Effect of glycine propionyl-L-carnitine on aerobic and ... Results indicated that power output was up to 15% higher following the glycine propionyl-l-carnitine trials compared to placebo ... Smith, WA; Fry, AC; Tschume, LC; Bloomer, RJ (2008). "Effect of glycine propionyl-L-carnitine on aerobic and anaerobic exercise ...
In enzymology, a (S)-carnitine 3-dehydrogenase (EC 1.1.1.254) is an enzyme that catalyzes the chemical reaction (S)-carnitine ... The systematic name of this enzyme class is (S)-carnitine:NAD+ oxidoreductase. Setyahadi S, Ueyama T, Arimoto T, Mori N, ... carnitine and NAD+, whereas its 3 products are 3-dehydrocarnitine, NADH, and H+. This enzyme belongs to the family of ... Kitamoto Y (1997). "Purification and properties of a new enzyme, D-carnitine dehydrogenase, from Agrobacterium sp. 525a". ...
Primary carnitine deficiency Carnitine palmitoyltransferase I deficiency Carnitine palmitoyltransferase II deficiency Reference ... Carnitine, a natural substance acquired mostly through the diet, is used by cells to process fats and produce energy. People ... Carnitine-acylcarnitine translocase deficiency is a rare, autosomal recessive metabolic disorder that prevents the body from ... Free long-chain fatty acids or those that are joined with carnitine can affect the electrical properties of cardiac cells ...
The co-A is then exchanged with carnitine (via the enzyme carnitine palmitoyltransferase I) to produce a fatty acid-carnitine ... Once inside, carnitine is liberated (catalysed by the enzyme carnitine palmitoyltransferase II) and transported back outside so ... Meldonium has also been shown by NMR to bind to carnitine acetyltransferase. Carnitine acetyltransferase belongs to a family of ... Carnitine is mainly absorbed from the diet, but can be formed through biosynthesis. To produce carnitine, lysine residues are ...
Carnitine biosynthesis γ-Butyrobetaine hydroxylase 4-N-Trimethylaminobutyraldehyde dehydrogenase Hulse JD, Ellis SR, Henderson ... "Where Does Nε-Trimethyllysine for the Carnitine Biosynthesis in Mammals Come from?". PLoS ONE. 9 (1): e84589. doi:10.1371/ ... This enzyme participates in lysine degradation and L-carnitine biosynthesis and requires the presence of iron and ascorbate. ... LM (1978). "Carnitine biosynthesis. beta-Hydroxylation of trimethyllysine by an alpha-ketoglutarate-dependent mitochondrial ...
The enzymes responsible for the degradation of carnitine to 3-dehydrocarnitine are carnitine-3-dehydrogenase or (S)-carnitine-3 ... The L-carnitine is metabolized at its beta-hydroxy group by L-carnitine-3-dehydrogenase and the coenzyme NAD+, which forms 3- ... 3-Dehydrocarnitine has a role as a human metabolite, as it is an intermediate of the degradation of carnitine. Carnitine is ... Carnitine Carnitine dehydrogenase Enzyme Metabolism Pseudomonas PubChem. "3-Dehydrocarnitine". pubchem.ncbi.nlm.nih.gov. ...
Vitamin B20: L-carnitine. Vitamin Bf: carnitine. Vitamin Bm: myo-inositol, also called "mouse antialopaecia factor". Vitamin Bp ... Vitamin BT: carnitine. Vitamin Bv: a type of B6 other than pyridoxine. Vitamin BW: a type of biotin other than d-biotin. ... Carnitine is an essential dietary nutrient for certain worms, but not for humans. Vitamin B8: adenosine monophosphate (AMP), ... Vitamin B4: can refer to the distinct chemicals choline, adenine, or carnitine. Choline is synthesized by the human body, but ...
Carnitine palmitoyltransferase II deficiency (also known as CPT-II deficiency) leads to an excess long chain fatty acids, as ... "Carnitine plamitoyltransferase I deficiency". Genetics Home Reference. National Institute of Health. Retrieved 4 November 2013 ... Weiser, Thomas (1993). "Carnitine Palmitoyltransferase II Deficiency". NIH. PMID 20301431. Retrieved 22 November 2013. " ... Treatment generally includes dietary modifications and carnitine supplements. Galactosemia results from an inability to process ...
... a substandard diet lacking sufficient carnitine and lysine can lead to decreased carnitine levels, which can have significant ... Carnitine is synthesised from trimethyllysine, which is a product of the degradation of certain proteins, as such lysine must ... Finally, lysine has been shown to be a precursor for carnitine, which transports fatty acids to the mitochondria, where they ... Flanagan JL, Simmons PA, Vehige J, Willcox MD, Garrett Q (April 2010). "Role of carnitine in disease". Nutrition & Metabolism. ...
Yüce A, Demir H, Temizel IN, Koçak N (2004). "Serum carnitine and selenium levels in children with celiac disease". Indian ... Blood factors Carnitine - Deficiency. Prolactin - Deficiency (childhood). homocysteine - Excess. Megaloblastic anemia (MA) is ...
Eg; On average 63% of excess choline, and 31% of carnitine (+-13%) are converted to TMA by an average gut profile - even if ... Reducing the consumption of foods and supplements that contain carnitine, such as red meat (beef, lamb and pork), liver, and ... A fish-like body odor can result from extreme excess consumption of TMA precursors such as choline, carnitine and betaine ( ... When a food substance, supplement or medicine that contains a TMA precursor (choline or carnitine) is ingested. Some precursor ...
PRKAR1A Carnitine deficiency, systemic primary; 212140; SLC22A5 Carotid intimal medial thickness 1; 609338; PPARG Carpal tunnel ...
... and L-carnitine therapy in those with low carnitine levels. Avoidance of isoleucine overload might prevent neurological ... Carnitine supplementation may be helpful. Dialysis is effective but usually not necessary. Unconscious patients and those with ...
Its treatment is carnitine replacement. Urinary tract infection caused by urease-producing organisms (Proteus, Pseudomonas ... Medication-induced hyperammonemia can occur with valproic acid overdose, and is due to a deficiency in carnitine. ... Methylmalonic acidemia Isovaleric acidemia Propionic acidemia Carnitine palmitoyltransferase II deficiency Transient ...
Sinclair, Christopher; Gilchrist, James M.; Hennessey, James V.; Kandula, Manju (September 2005). "Muscle carnitine in hypo- ...
... taurine and carnitine supplementation may be indicated even if the dog does not have a documented taurine or carnitine ... Sanderson SL (November 2006). "Taurine and carnitine in canine cardiomyopathy". The Veterinary Clinics of North America. Small ... deficiency although the cost of carnitine supplementation may be viewed as prohibitive by some Dilated cardiomyopathy is also a ...
L-Carnitine, acetyl-l-carnitine, and propionyl-l-carnitine are available in dietary supplement pills or powders, with a daily ... Carnitine exists as one of two stereoisomers (the two enantiomers d-carnitine (S-(+)-) and l-carnitine (R-(−)-)). Both are ... Two types of carnitine deficiency states exist. Primary carnitine deficiency is a genetic disorder of the cellular carnitine- ... Even carnitine-poor diets have little effect on total carnitine content, as the kidneys conserve carnitine. In general, ...
Primary carnitine deficiency is a condition that prevents the body from using certain fats for energy, particularly during ... Longo N, Amat di San Filippo C, Pasquali M. Disorders of carnitine transport and the carnitine cycle. Am J Med Genet C Semin ... Carnitine transport by organic cation transporters and systemic carnitine deficiency. Mol Genet Metab. 2001 Aug;73(4):287-97. ... Amat di San Filippo C, Pasquali M, Longo N. Pharmacological rescue of carnitine transport in primary carnitine deficiency. Hum ...
Carnitine is a naturally occurring hydrophilic amino acid derivative, produced endogenously in the kidneys and liver and ... Primary carnitine deficiency is caused by a deficiency in the plasma membrane carnitine transporter, with urinary carnitine ... Carnitine deficiency is a metabolic state in which carnitine concentrations in plasma and tissues are less than the levels ... Longo N, Amat di San Filippo C, Pasquali M. Disorders of carnitine transport and the carnitine cycle. Am J Med Genet C Semin ...
Seemingly at odds with a recent report implicating L-carnitine as a possible villain in heart disease, a report points to a ... "But if carnitine is the problem, certainly high carnitine would be worse than low carnitine," proposed DiNicolantonio. The ... Relative Risk (95% CI) for Outcomes After Treatment of Acute MI With L-Carnitine, Meta-analysis ... But were pretty certain that in the acute setting going out to up to a year, L-carnitine had no signal of harm in any of the ...
The (,stereo,S,/stereo,)-enantiomer of carnitine. ChEBI CHEBI:11060. The (S)-enantiomer of carnitine. ChEBI https://www.ebi.ac. ...
Find Carnitine supplements at Thorne. Ingredients backed by science and research, in forms your body can actually absorb. ...
L-carnitine is a naturally occurring amino acid derivative thats often taken as a weight loss supplement. It has several ... Here are several other types of carnitine:. *D-carnitine: This inactive form has been shown to reduce blood levels of carnitine ... L-carnitine may be beneficial for people with type 2 diabetes.. A recent review of 41 studies concluded that L-carnitine ... This makes L-carnitine a conditionally essential nutrient (. 2. ).. Different types. L-carnitine is the standard biologically ...
L-Carnitine is a non-essential amino acid that helps to maintain overall good health by facilitating the transfer of fatty acid ... NOW Foods, L-Carnitine, 500 mg, 180 Veg Capsules By. NOW Foods. ... L-Carnitine (Carnipure™). (from 746 mg L-Carnitine Tartrate). ... NOW® L-Carnitine is the purest form, clinically tested, and vegetarian (non-animal sourced). ...
Because of this, the body produces insufficient amounts of carnitine. Carnitine is important because it helps transport fatty ... He sees carnitine supplements in the same light as folic acid, which poses so little risk and carries such an upside that the ... Carnitine, which is available as an over-the-counter nutritional supplement and is found in red meats and whole milk, could ... Lack of carnitine appears to result in a fetus neural stem cells not getting fatty acid to the correct places, increasing ...
Acyl-carnitine is shuttled inside by a carnitine-acylcarnitine translocase. *Acyl-carnitine is converted to acyl-CoA by ... Carnitine, also known as L-carnitine or levocarnitine, is a quaternary ammonium compound biosynthesized from the amino acids ... carnitine palmitoyltransferase I deficiency, carnitine palmitoyltransferase II deficiency and carnitine-acylcarnitine ... Carnitine was originally found as a growth factor for mealworms and labeled vitamin Bt. Natural carnitine is the L-stereoisomer ...
Carnitine-acylcarnitine translocase deficiency. Disease definition Carnitine-acylcarnitine translocase (CACT) deficiency is a ... Free carnitine is very low. Specific enzyme analysis in cultured fibroblasts or lymphocytes will confirm a diagnosis as will ... Carnitine supplementation is also recommended. During an acute episode, intravenous glucose is administered in order to inhibit ... It is an essential component of the carnitine cycle that regulates the transport of long chain fatty acids into the ...
Carnitine is a naturally occurring hydrophilic amino acid derivative, produced endogenously in the kidneys and liver and ... Carefully monitor adequate carnitine dose in primary and secondary carnitine deficiencies by evaluating plasma carnitine levels ... Primary carnitine deficiency. Patients with primary carnitine deficiency have excellent prognosis with oral carnitine ... Provide intravenous (IV) carnitine if the patient is known to have carnitine deficiency and a defect affecting the oxidation of ...
Acetyl-L-carnitine was found to reduce total 14CO2 release from [U-14C]glucose along with the decrease in [1 … ... The effects of acetyl-L-carnitine on cerebral glucose metabolism were investigated in rats injected with differently 14C- and ... Acetyl-L-carnitine modulates glucose metabolism and stimulates glycogen synthesis in rat brain Brain Res. 1998 Jun 15;796(1-2): ... Acetyl-L-carnitine was found to reduce total 14CO2 release from [U-14C]glucose along with the decrease in [1-13C]glucose ...
You should not use the information on this site for diagnosis or treatment of any health problem or for prescription of any medication or other treatment. Always seek the advice of your physician or other qualified health care provider with any questions you may have regarding a medical condition. You should consult with a health care professional before starting any diet, exercise, or supplementation program, before taking any medication, or if you have or suspect you might have a health problem. ...
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Natures Best L-Carnitine is a high-strength, UK-made supplement. It is popular with active individuals and those engaged in ... To ensure Carnitine 500mg meets our strict quality standards, we manufacture and package our high-strength formula in UK ... Want to learn more about Carnitine? Drop a line to one of our expert Nutrition Advisors, who are always happy to provide free, ... Typically found in red meat, the amino acid, L-Carnitine, contributes to the release of energy from food. It continues to be a ...
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Heart and skeletal muscle contain abundant amounts of L-carnitine, which is essential for meeting tissue energy demands by ... pure L-carnitine produced according to USP guidelines and are free of potentially harmful synthetic D-carnitine.. ... L-Carnitine supplementation can support liver, blood sugar and cholesterol metabolism, and cardiac function.† These capsules ... Heart and skeletal muscle contain abundant amounts of L-carnitine, which is essential for meeting tissue energy demands by ...
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Carnitine is a naturally occurring hydrophilic amino acid derivative, produced endogenously in the kidneys and liver and ... Primary carnitine deficiency is caused by a deficiency in the plasma membrane carnitine transporter, with urinary carnitine ... Carnitine deficiency is a metabolic state in which carnitine concentrations in plasma and tissues are less than the levels ... Longo N, Amat di San Filippo C, Pasquali M. Disorders of carnitine transport and the carnitine cycle. Am J Med Genet C Semin ...
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  • Some individuals with genetic or medical disorders (such as preterm infants) cannot make enough carnitine, requiring dietary supplementation. (wikipedia.org)
  • One review of 37 studies found that L-carnitine supplementation significantly reduced body weight, body mass index (BMI), and fat mass. (healthline.com)
  • Ensure uninterrupted carnitine supplementation. (medscape.com)
  • Patients with primary carnitine deficiency have excellent prognosis with oral carnitine supplementation. (medscape.com)
  • Hypoglycemia or sudden deaths from arrhythmias (even without cardiomyopathy) have been reported in patients who stop their carnitine supplementation against medical advice. (medscape.com)
  • L-Carnitine supplementation can support liver, blood sugar and cholesterol metabolism, and cardiac function. (fullscript.com)
  • In a 21-day human, clinical, placebo-controlled study that was later published in a leading American physiological journal, L-Carnitine supplementation supported muscle tissue regeneration. (bodybuilding.com)
  • L-Carnitine L-Tartrate Supplementation Favorable Affects Markers of Recovery from Exercise Stress. (bodybuilding.com)
  • 15-17 Fortunately, carnitine supplementation has proven to be remarkably effective in fighting and even reversing the heart-weakening effects from that drop in carnitine levels. (lifeextension.com)
  • L-carnitine supplementation also prevents the progression of heart muscle damage in people with congestive heart failure and improves exercise tolerance in people who develop chest pain (angina) with exertion. (lifeextension.com)
  • Research found supplementation with Acetyl L-Carnitine helped to reduce mitochondrial decay (NIH 2021). (professionalwhey.com.au)
  • Long term supplementation of l-carnitine can reduce the detrimental effects of aging on one's body. (womanjunction.com)
  • L-carnitine supplementation wasn't toxic, some patients seemed to improve on it. (pallimed.org)
  • Mechanisms underlying the anti-wasting effect of L-carnitine supplementation under pathologic conditions: evidence from experimental and clinical studies. (arnoldsupplements.com)
  • Effects of acute L-carnitine supplementation on nitric oxide production and oxidative stress after exhaustive exercise in young soccer players. (arnoldsupplements.com)
  • L-Carnitine naturally occurs in red meat and other animal source foods, but we recommend supplementation to obtain optimal levels of this excellent amino acid. (nourishingfoods.com)
  • Effects of parenteral L-carnitine supplementation on fat metabolism and nutrition in premature neonates. (medscape.com)
  • His mental state improved dramatically after starting L-carnitine supplementation (600mg twice daily). (bvsalud.org)
  • Primary carnitine deficiency is a condition that prevents the body from using certain fats for energy, particularly during periods without food (fasting). (medlineplus.gov)
  • Signs and symptoms of primary carnitine deficiency typically appear during infancy or early childhood and can include severe brain dysfunction (encephalopathy), a weakened and enlarged heart (cardiomyopathy), confusion, vomiting, muscle weakness, and low blood glucose (hypoglycemia). (medlineplus.gov)
  • Some people with primary carnitine deficiency are asymptomatic, which means they do not have any signs or symptoms of the condition. (medlineplus.gov)
  • Problems related to primary carnitine deficiency can be triggered by periods of fasting or by illnesses such as viral infections. (medlineplus.gov)
  • The incidence of primary carnitine deficiency in the general population is approximately 1 in 100,000 newborns. (medlineplus.gov)
  • Mutations in the SLC22A5 gene cause primary carnitine deficiency. (medlineplus.gov)
  • As a result, there is a shortage (deficiency) of carnitine within cells. (medlineplus.gov)
  • Reduced energy production can lead to some of the features of primary carnitine deficiency, such as muscle weakness and hypoglycemia. (medlineplus.gov)
  • Amat di San Filippo C, Pasquali M, Longo N. Pharmacological rescue of carnitine transport in primary carnitine deficiency. (medlineplus.gov)
  • Maternal systemic primary carnitine deficiency uncovered by newborn screening: clinical, biochemical, and molecular aspects. (medlineplus.gov)
  • Carnitine transport by organic cation transporters and systemic carnitine deficiency. (medlineplus.gov)
  • Carnitine deficiency is a metabolic state in which carnitine concentrations in plasma and tissues are less than the levels required for normal function of the organism. (medscape.com)
  • Carnitine deficiency may be primary or secondary. (medscape.com)
  • Primary carnitine deficiency is caused by a deficiency in the plasma membrane carnitine transporter, with urinary carnitine wasting causing systemic carnitine depletion. (medscape.com)
  • [ 1 ] Intracellular carnitine deficiency impairs the entry of long-chain fatty acids into the mitochondrial matrix. (medscape.com)
  • Muscle carnitine deficiency (restricted to muscle) is characterized by depletion of carnitine levels in muscle with normal serum concentrations. (medscape.com)
  • In secondary carnitine deficiency, which is caused by other metabolic disorders (eg, fatty acid oxidation disorders, organic acidemias), carnitine depletion may be secondary to the formation of acylcarnitine adducts and the inhibition of carnitine transport in renal cells by acylcarnitines. (medscape.com)
  • Preterm newborns also may be at risk for developing carnitine deficiency because immature renal tubular function combined with impaired carnitine biosynthesis renders them strictly dependent on exogenous supplies to maintain normal plasma carnitine levels. (medscape.com)
  • Valproic acid may cause an acquired type of secondary carnitine deficiency by directly impairing renal tubular reabsorption of carnitine. (medscape.com)
  • In a Japanese study, primary systemic carnitine deficiency was estimated to occur in 1 per 40,000 births. (medscape.com)
  • In order to abate the mortality and morbidity of undiagnosed primary carnitine deficiency, this condition has been included in the expanded newborn screening program in several states within the United States. (medscape.com)
  • Dysfunction of this process leads to the genetic disorders primary carnitine deficiency , carnitine palmitoyltransferase I deficiency , carnitine palmitoyltransferase II deficiency and carnitine-acylcarnitine translocase deficiency . (wikidoc.org)
  • Carnitine-acylcarnitine translocase (CACT) deficiency is a life-threatening, inherited disorder of fatty acid oxidation which usually presents in the neonatal period with severe hypoketotic hypoglycemia, hyperammonemia, cardiomyopathy and/or arrhythmia, hepatic dysfunction, skeletal muscle weakness, and encephalopathy. (orpha.net)
  • The neonatal and severe infantile forms of carnitine palmitoyl transferase II (CPT II) deficiency (see this term) need to be excluded as they have an identical acylcarnitine profile to CACT. (orpha.net)
  • Carefully review diet compliance in secondary carnitine deficiency, considering avoidance of fasting, intake of fat-restricted, high-carbohydrate diet, and other dietary supplements that may be needed, such as riboflavin or glycine. (medscape.com)
  • Admit patients with carnitine deficiency for medical management of acute metabolic decompensation. (medscape.com)
  • Provide intravenous (IV) carnitine if the patient is known to have carnitine deficiency and a defect affecting the oxidation of long chain fatty acids has been excluded. (medscape.com)
  • Medications include carnitine for primary and secondary carnitine deficiency, as well as other cofactors that may be needed for different conditions associated with secondary carnitine deficiency (eg, riboflavin, coenzyme Q, biotin, hydroxocobalamin, betaine, glycine). (medscape.com)
  • Avoid exercise and dehydration with warm temperatures because attacks of rhabdomyolysis may occur with certain conditions that cause secondary carnitine deficiency. (medscape.com)
  • Prognosis of secondary carnitine deficiency depends on the nature of the disorder. (medscape.com)
  • Translocase deficiency and the infantile form of carnitine palmitoyltransferase II (CPT-II) deficiency have very poor prognosis regardless of treatment. (medscape.com)
  • Other metabolic disorders that cause secondary carnitine deficiency, such as organic acidemias, require lifelong diet modification and nutritional supplements. (medscape.com)
  • Family members should receive education once the work-up initiated after newborn screening results suggests primary carnitine deficiency in the newborn or in the mother. (medscape.com)
  • Simply put, a carnitine deficiency leads to the wholesale destruction of our mitochondria. (lifeextension.com)
  • However, those with genetic abnormalities or other health conditions causing deficiency of L-carnitine should talk to their doctor for a more specific dosage. (healthproductsforyou.com)
  • L-carnitine deficiency is often associated with age, since as we advance in age our mitochondrial functions decline, causing a decline in energy production. (womanjunction.com)
  • Carnitine deficiency results from inadequate intake of or inability to metabolize the amino acid carnitine. (msdmanuals.com)
  • Symptoms of carnitine deficiency and the age at which symptoms appear depend on the cause. (msdmanuals.com)
  • In neonates, carnitine palmitoyltransferase deficiency is diagnosed using mass spectrometry to screen blood. (msdmanuals.com)
  • Carnitine deficiency due to inadequate dietary intake, increased requirements, excess losses, decreased synthesis, or (sometimes) enzyme deficiencies can be treated by giving L-carnitine 25 mg/kg orally every 6 hours. (msdmanuals.com)
  • Systemic primary carnitine deficiency: an overview of clinical manifestations, diagnosis, and management. (medscape.com)
  • Genetic epidemiology of the carnitine transporter OCTN2 gene in a Japanese population and phenotypic characterization in Japanese pedigrees with primary systemic carnitine deficiency. (medscape.com)
  • Expanded newborn screening identifies maternal primary carnitine deficiency. (medscape.com)
  • Pericardial effusion in primary systemic carnitine deficiency. (medscape.com)
  • Agnetti A, Bitton L, Tchana B, Raymond A, Carano N. Primary carnitine deficiency dilated cardiomyopathy: 28years follow-up. (medscape.com)
  • Carnitine palmitoyltransferase-1b deficiency aggravates pressure overload-induced cardiac hypertrophy caused by lipotoxicity. (medscape.com)
  • Angelini C, Vergani L, Martinuzzi A. Clinical and biochemical aspects of carnitine deficiency and insufficiency: transport defects and inborn errors of beta-oxidation. (medscape.com)
  • Primary and secondary carnitine deficiency syndromes. (medscape.com)
  • Carnitine deficiency presenting with a decreased mental state in a patient with amyotrophic lateral sclerosis receiving long-term tube feeding: a case report. (bvsalud.org)
  • This case highlights the importance of avoiding carnitine deficiency in patients with amyotrophic lateral sclerosis undergoing long-term tube feeding . (bvsalud.org)
  • Carnitine deficiency can be misdiagnosed as a progression of systemic muscle atrophy . (bvsalud.org)
  • In a red-letter week for L-carnitine, an amine derived in vivo primarily from two amino acids and frequently included in dietary supplements and commercial energy drinks, two journals have separately released a pair of studies with what may seem contradictory conclusions. (medscape.com)
  • On April 7, Nature Medicine published a prospective series of experiments in mice and human subjects that together suggest, according to the authors, that high levels of dietary carnitine, especially in red meat but also in supplements, may indirectly accelerate atherosclerosis . (medscape.com)
  • L-carnitine is an amino acid produced by the body that's also found in food and supplements. (healthline.com)
  • This article examines the potential risks and benefits of L-carnitine supplements and explains how this nutrient functions in your body. (healthline.com)
  • L-carnitine is the standard biologically active form of carnitine, which is found in your body, foods, and most supplements. (healthline.com)
  • He sees carnitine supplements in the same light as folic acid, which poses so little risk and carries such an upside that the Food and Drug Administration allowed it to be added to many foods without extensive clinical trials. (heraldnet.com)
  • L -Carnitine products and supplements are not allowed to be imported into Canada( Health Canada ). (wikidoc.org)
  • Although our bodybuilding forefathers were as dedicated to the sport, gyms were nothing short of iron dungeons, and supplements like L-Carnitine were non-existent. (bodybuilding.com)
  • Question: On a recent vacation in Florida, I placed up to a month's supply of my supplements in a daily pillbox and my Swanson Premium Brand L-Carnitine tablets turned the compartments into a mess. (swansonvitamins.com)
  • The U.S. FDA posted a Warning Letter to CJ Labs (Miami, Florida) dated November 1, 2011 regarding serious violations of Good Manufacting Practices in the production of its Milk Thistle and L-Carnitine supplements. (consumerlab.com)
  • HPFY offers L-carnitine supplements from top brands at affordable prices. (healthproductsforyou.com)
  • Consuming the best l-carnitine supplements can positively affect overall well-being and improve your training results. (healthproductsforyou.com)
  • When to consume L-Carnitine Supplements? (healthproductsforyou.com)
  • L-carnitine supplements are often used to remedy low levels of the natural amino acid discovered in women. (womanjunction.com)
  • Many women who have used l-carnitine supplements have reported unusual changes or problems affecting their menstrual cycles. (womanjunction.com)
  • On the contrary, taking l-carnitine supplements can also start up a period that has been very light or absent. (womanjunction.com)
  • Most people don't need to get their intake of l-carnitine from food or supplements as the body is supposed to naturally produce enough on its own to meet its necessary needs. (womanjunction.com)
  • Having enough l-carnitine produced in the body is important, and if you decide to begin taking supplements to support your health you should first consult with a doctor for advice due to the nature of its side effects. (womanjunction.com)
  • L-carnitine supplements can be taken through tablets, capsules, and powder. (womanjunction.com)
  • While taking l-carnitine can certainly boost your health, exercise performance, and fat burning, increased amounts of l-carnitine whether received through supplements or food can cause side effects, including changes in one's menstrual cycle. (womanjunction.com)
  • Supplements containing L-carnitine can NOT be shipped to Canada due to Canadian laws. (priceplow.com)
  • In support of energy metabolism, carnitine transports long-chain fatty acids from the cytosol into mitochondria to be oxidized for free energy production, and also participates in removing products of metabolism from cells. (wikipedia.org)
  • This transesterification is catalyzed by an enzyme found in the outer membrane of the mitochondria known as carnitine acyltransferase 1 (also called carnitine palmitoyltransferase 1, CPT1). (wikipedia.org)
  • Without carnitine, fatty acids cannot enter mitochondria and be used to make energy. (medlineplus.gov)
  • The group speculates that the short- and longer-term CV benefits relate primarily to the ability of L-carnitine to promote elimination of toxic fatty-acid metabolites and facilitate the transport of long-chain fatty acids into the mitochondria, improving glucose oxidation. (medscape.com)
  • It is an essential component of the carnitine cycle that regulates the transport of long chain fatty acids into the mitochondria where fatty acid oxidation takes place. (orpha.net)
  • Heart and skeletal muscle contain abundant amounts of L-carnitine, which is essential for meeting tissue energy demands by transporting fatty acids to the mitochondria. (fullscript.com)
  • Carnitine is an amino acid that transports lipids into the mitochondria where they are oxidized for energy. (naturalremedies.org)
  • Total carnitine levels in aging individuals are roughly 20% lower than in youth, 11 which leaves mitochondria increasingly vulnerable to damage. (lifeextension.com)
  • This is a unique property of Acetyl L-Carnitine as current research indicates it enhances the metabolism of cellular health in the brain and central nervous system (CNS) via mitochondria. (professionalwhey.com.au)
  • L-carnitine transfers long-chain fatty acids, such as triglycerides into mitochondria, where they may be oxidized to produce energy. (supervits.com)
  • The amino acid carnitine is required for the transport of long-chain fatty acyl coenzyme A (CoA) esters into myocyte mitochondria, where they are oxidized for energy. (msdmanuals.com)
  • Acetylcarnitine is broken down in the blood by plasma esterases to carnitine which is used by the body to transport fatty acids into the mitochondria for breakdown. (aksci.com)
  • During strenuous exercise, a large portion of L-carnitine and unused acetyl-CoA are converted to ALCAR and CoA inside mitochondria by carnitine O-acetyltransferase.The ALCAR is transported outside the mitochondria where it converts back to the two constituents. (aksci.com)
  • The L-carnitine is cycled back into the mitochondria with acyl groups to facilitate fatty acid utilization, but excess acetyl-CoA may block it.Excess acetyl-CoA causes more carbohydrates to be used for energy at the expense of fatty acids. (aksci.com)
  • ALCAR transport decreases acetyl-CoA inside the mitochondria, but increases it outside.Glucose metabolism in diabetics improves with administration of either ALCAR or L-carnitine. (aksci.com)
  • L-carnitine transports long-chain fatty acids across the mitochondrial membrane to be metabolized. (priceplow.com)
  • Carnitine is involved in transporting fatty acids across the mitochondrial membrane, by forming a long chain acetylcarnitine ester and being transported by carnitine palmitoyltransferase I and carnitine palmitoyltransferase II. (wikipedia.org)
  • Carnitine is a quaternary ammonium compound involved in metabolism in most mammals, plants, and some bacteria. (wikipedia.org)
  • The apparent survival gains from L-carnitine, which were only modestly significant, seemed to come from infarct-size limitation and cardiomyocyte membrane stabilization, with chronic improvements in cellular energy metabolism, according to their report published online today in the Mayo Clinic Proceedings . (medscape.com)
  • The effects of acetyl-L-carnitine on cerebral glucose metabolism were investigated in rats injected with differently 14C- and 13C-labelled glucose and sacrificed after 15, 30, 45, and 60 min. (nih.gov)
  • Acetyl L-Carnitine (ALC) is a nutrient and naturally occurring metabolite in the human body that is involved in lipid, carbohydrate, and protein metabolism. (bayho.com)
  • TMAO is a product of carnitine metabolism by microbiota in the gut. (naturalmedicinejournal.com)
  • Intestinal microbiota metabolism of L-carnitine, a nutrient in red meat, promotes atherosclerosis. (naturalmedicinejournal.com)
  • Top Secret Nutrition's L-Carnitine Plus Green Coffee Extract is a synergistic fat burning support blend, designed to boost energy and metabolism while promoting fat loss. (priceplow.com)
  • L-Carnitine is a naturally occurring amino acid which plays a vital role in promoting the metabolism of fat. (priceplow.com)
  • Top Secret Nutrition s FIREBALL L-Carnitine Liquid Igniter is designed to boost your metabolism, burn fat, create thermogenesis, and improve exercise performance. (arnoldsupplements.com)
  • doi: 10.1113/jphysiol.2010.201343 Chronic oral ingestion of l-carnitine and carbohydrate increases muscle carnitine content and alters muscle fuel metabolism during exercise in humans. (arnoldsupplements.com)
  • L-Carnitine is a naturally occurring amino acid which plays a vital role in the metabolism of fat. (vimandvigor.com)
  • Acetyl-L-carnitine is also used as a dietary supplement as a more orally available precursor to L-carnitine in regards to carbohydrate and fat metabolism. (aksci.com)
  • De Vivo D, Tein I. Primary and secondary disorders of carnitine metabolism. (medscape.com)
  • Metabolic pathway enrichment identified 41 pathways associated with benzene exposure, with altered pathways including carnitine shuttle, fatty acid metabolism, sulfur amino acid metabolism, glycolysis, gluconeogenesis and branched chain amino acid metabolism. (cdc.gov)
  • RELOADED: with 3000mg Triple Action L-Carnitine (99% PUre Base, Tartrate, Acetyl), 25mg GBB Carnitine Precursor, 15mg ParaBurn Grains of Paradise and 5mg of Bioperine Black Pepper Extract (95% piperine) in each serving! (allstarhealth.com)
  • Note: To avoid confusion we recommmend our L-Carnitine L-Tartrate for superior fat burning, recovery and performance benefits. (professionalwhey.com.au)
  • Two studies led by William J. Kraemer tested the hormonal response to taking carnitine tartrate. (supervits.com)
  • L-Carnitine Tartrate l-carnitine is a dipeptide made from the essential amino acids lysine and methionine. (arnoldsupplements.com)
  • Tartrate is an organic compound used to improve absorption l-carnitine. (arnoldsupplements.com)
  • Carnitine exists as one of two stereoisomers (the two enantiomers d-carnitine (S-(+)-) and l-carnitine (R-(−)-)). Both are biologically active, but only l-carnitine naturally occurs in animals, and d-carnitine is toxic as it inhibits the activity of the l-form. (wikipedia.org)
  • L-Carnitine is the biologically active strain of the amino acid. (naturalremedies.org)
  • Each easy-to-swallow, vegan-friendly capsule supplies an impressive 500mg of Carnitine in its L-form, not synthetic D form, allowing for superior absorption. (naturesbest.co.uk)
  • To ensure Carnitine 500mg meets our strict quality standards, we manufacture and package our high-strength formula in UK factories working to rigorous Good Manufacturing Practice (GMP). (naturesbest.co.uk)
  • Acetyl L-Carnitine usually comes in doses of 500mg or 250mg. (dinet.org)
  • View cart "Fertility Restore Acetyl L-Carnitine 500mg (30 Caps)" has been added to your cart. (drmyattswellnessclub.com)
  • Among other measuring techniques, researchers used magnetic resonance imaging (MRI), to test the effects of a 2 gram per day dose of L-Carnitine, enough to maximize plasma carnitine concentration, on a group of healthy, recreationally weight-trained men. (bodybuilding.com)
  • Despite common carnitine supplement consumption among athletes for improved exercise performance or recovery, there is insufficient high-quality clinical evidence to indicate it provides any benefit. (wikipedia.org)
  • L-carnitine is a naturally occurring amino acid derivative that's often taken as a supplement. (healthline.com)
  • L-carnitine is a nutrient and dietary supplement. (healthline.com)
  • Because L-carnitine helps move more fatty acids into your cells to be burned for energy, it's sometimes used as a weight loss supplement . (healthline.com)
  • Carnitine, which is available as an over-the-counter nutritional supplement and is found in red meats and whole milk, could play a key role in preventing some forms of autism from developing during the early days of pregnancy. (heraldnet.com)
  • There's not a consensus on whether pregnant women should supplement their carnitine intake, but the A&M researchers see it as a step with virtually no downside if done under medical supervision, comparing it to the widely accepted recommendation that pregnant women consume folic acid, which is added to many foods because it helps prevent defects in a baby's brain and spinal cord. (heraldnet.com)
  • For people considering starting a family who are on, say, a vegan diet that has little carnitine, "I would ask my doctor if I could take a corrective supplement," Bankaitis said. (heraldnet.com)
  • By now, you shouldn't need any further convincing that L-Carnitine is among the most important elements in any serious bodybuilder's supplement program. (bodybuilding.com)
  • I wanted to let everyone know about a supplement called Acetyl L-Carnitine. (dinet.org)
  • Fortunately, carnitine is sold as a dietary supplement in the United States, available without the need of a doctor's prescription as it is in some other countries. (lifeextension.com)
  • For athletes, regardless of what your bodybuilding and fitness goals are, losing fat or gaining muscle, your chances of success will increase greatly if you have l-carnitine in your supplement arsenal. (supervits.com)
  • Researchers gave a carnitine supplement to mice who were fed a high-fat diet in order to make them gain weight. (supervits.com)
  • Some medical professionals recommend L-carnitine in supplement form to help support those with diabetes, heart conditions, erectile dysfunction, and other health conditions. (healthproductsforyou.com)
  • You can break up the prescribed daily dose of L-carnitine supplement into two or three doses as per your comfort and take it with a high carbohydrate, high protein meal throughout the day. (healthproductsforyou.com)
  • Palmitoyl-L-carnitine-d3 Hydrochloride is labelled Palmitoyl-L-carnitine Hydrochloride (P155050), a long-chain fatty ester of carnitine which is accumulated and released into the circulation, in fatty-acid oxidation defects. (trc-canada.com)
  • ALC is special form of Carnitine that helps the body produce energy, and healthy brains also require a lot of it to function at their peak. (bronsonvitamins.com)
  • Generally individuals, including strict vegetarians, synthesize enough L-carnitine in vivo. (wikipedia.org)
  • [2] Vitamin C ( ascorbic acid ) is essential to the synthesis of carnitine. (wikidoc.org)
  • Carnitine also plays a role in stabilizing Acetyl-CoA and coenzyme A levels through the ability to receive or give an acetyl group. (wikipedia.org)
  • For most people, acetyl-L-carnitine and L-carnitine seem to be the most effective for general use. (healthline.com)
  • Some research suggests that the acetyl form, acetyl-L-carnitine (ALCAR), may help prevent age-related mental decline and improve markers of learning ( 13 ). (healthline.com)
  • Acetyl-L-carnitine was found to reduce total 14CO2 release from [U-14C]glucose along with the decrease in [1-13C]glucose incorporation into cerebral amino acids and tricarboxylic acid cycle intermediates. (nih.gov)
  • However the 13C labelling pattern within different carbon positions of glutamate, glutamine, GABA, and aspartate was unaffected by acetyl-L-carnitine administration. (nih.gov)
  • Furthermore, the cerebral levels of newly-synthesized proglycogen were higher in rats treated with acetyl-L-carnitine than in untreated ones. (nih.gov)
  • These results suggest that acetyl-L-carnitine was able to modulate cerebral glucose utilization and provide new insights on the mechanisms of action of this molecule in the central nervous system. (nih.gov)
  • Vital Nutrients Acetyl L-Carnitine supports normal acetylcholine levels, an important neurotransmitter. (acuatlanta.net)
  • Acetyl L-Carnitine product is available only from licensed healthcare professionals. (acuatlanta.net)
  • Buy Acetyl L-Carnitine Online here at AcuAtlanta.net or our clinic. (acuatlanta.net)
  • Whilst L-Carnitine L-Tartarte has been shown to be superior in assisting with fat loss, performance and recovery, Acetyl L-Carnitine is the neurological version of L-Carnitine that offers superior improvements in cognitive enhancement and neuroprotection over other forms of L-Carnitine. (professionalwhey.com.au)
  • Various other studies have shown that Acetyl L-Carnitine may have significant impact upon specific groups of people who may suffer from PTSD memory impairment (e.g. military and emergency services) and geriatric (elderly) cognitive disorders like dementia, depression and anxiety. (professionalwhey.com.au)
  • However Acetyl L-Carnitine has also been shown in some studies to be involved in supporting cardiovascular health. (professionalwhey.com.au)
  • Consume 2g of Acetyl L-Carnitine daily in 100ml of water. (professionalwhey.com.au)
  • Simply put Acetyl L Carnitine Alpha Lipoic Acid to work for you. (bronsonvitamins.com)
  • Acetyl L Carnitine Alpha Lipoic Acid offers a "dynamic duo" of antioxidants to shield against free radicals and maintain cell health. (bronsonvitamins.com)
  • The benefits of Acetyl L Carnitine (ALC) are remarkable, too. (bronsonvitamins.com)
  • Need even more Alpha Lipoic Acid Acetyl L Carnitine benefits? (bronsonvitamins.com)
  • Alpha Lipoic Acid Acetyl L Carnitine benefits the body when you add it to your daily health regimen. (bronsonvitamins.com)
  • Bluebonnet's Acetyl L-Carnitine 500 mg Vegetable Capsules are formulated with the free-form amino acid acetyl L-carnitine to help support cellular energy production. (bluebonnetnutrition.com)
  • Effect of Acetyl-L-carnitine on. (checkorphan.org)
  • Acetyl L-Carnitine is essentially a processed form of L-Carnitine that can pass the blood-brain barrier to help energize the brain. (arnoldsupplements.com)
  • Acetyl L-Carnitine (ALCAR) has backed claims of anti-aging and nutrient regenerating properties. (organicsonabudget.com.au)
  • Acetyl L-Carnitine is derived from the amino acids lysine and methionine. (organicsonabudget.com.au)
  • Acetyl L-Carnitine is an excellent training aid for energy, focus and weight loss. (organicsonabudget.com.au)
  • Acetyl-L-carnitine or ALCAR, is an acetylated form of L-carnitine (a betaine derivative of beta-hydroxybutyrate, is found in virtually all cells of higher animals and also in some microorganisms and plants. (aksci.com)
  • No authors listed] Acetyl-L-carnitine. (aksci.com)
  • We've chosen to encapsulate our high-performance product in capsules instead of tablets since this material can accommodate concentrated levels of pure-grade L-Carnitine. (naturesbest.co.uk)
  • Health Products For You offers l carnitine powder, l-carnitine capsules, and l-carnitine tablets from esteemed vendors like Life Extension , Finaflex , GAT , etc. (healthproductsforyou.com)
  • The tissue distribution of carnitine-biosynthetic enzymes in humans indicates TMLD to be active in the liver, heart, muscle, brain and highest in the kidneys. (wikipedia.org)
  • Carnitine is a naturally occurring hydrophilic amino acid derivative, produced endogenously in the kidneys and liver and derived from meat and dairy products in the diet. (medscape.com)
  • More than 95% of your L-carnitine stores are contained in your muscles, along with trace amounts in your blood, liver, heart, and kidneys ( 2 ). (healthline.com)
  • Carnitine is biosynthesized within the body from the amino acids lysine or methionine primarily in the liver and kidneys . (wikidoc.org)
  • The body uses lysine to create the amino acid carnitine in the liver and kidneys. (naturesbest.co.uk)
  • Synthesized in the liver and kidneys, L-Carnitine requires two essential amino acids, lysine and methionine, for synthesis to take place. (naturalremedies.org)
  • The primary source for L-Carnitine are the kidneys and liver, but they are not the only source of the amino acid. (naturalremedies.org)
  • The kidneys can store L-carnitine for later use and excrete the excess through the urine. (healthproductsforyou.com)
  • Carnitine , also known as L -carnitine or levocarnitine , is a quaternary ammonium compound biosynthesized from the amino acids lysine and methionine . (wikidoc.org)
  • Derived from amino acids, carnitine was first extracted from meat extracts in 1905, leading to its name from Latin, "caro/carnis" or flesh. (wikipedia.org)
  • Your body can produce L-carnitine out of the amino acids lysine and methionine ( 2 ). (healthline.com)
  • Before any discussion on Carnitine can take place, one must first understand the role of amino acids in the metabolic process. (naturalremedies.org)
  • In the second reaction, acyl-CoA is transiently attached to the hydroxyl group of carnitine to form fatty acylcarnitine. (wikipedia.org)
  • The fatty acylcarnitine ester formed then diffuses across the intermembrane space and enters the matrix by facilitated diffusion through carnitine-acylcarnitine translocase (CACT) located on the inner mitochondrial membrane. (wikipedia.org)
  • CACT is located in the inner mitochondrial membrane and operates a carnitine/acylcarnitine exchange across this membrane. (orpha.net)
  • Finally, in mice, it was observed that TMAO production in response to L-carnitine could be induced with microbial colonization in germ-free mice or with chronic dietary L-carnitine intake. (naturalmedicinejournal.com)
  • If you're looking to boost your l-carnitine intake for beneficial health reasons, it is recommended to take a range of two to four grams of l-carnitine daily [ 3 ]. (womanjunction.com)
  • A portion of L-carnitine is converted to ALCAR after ingestion in humans. (aksci.com)
  • These capsules contain 100% pure L-carnitine produced according to USP guidelines and are free of potentially harmful synthetic D-carnitine. (fullscript.com)
  • Gamma-butyrobetaine is then hydroxylated by gamma butyrobetaine hydroxylase (a zinc binding enzyme) into l-carnitine, requiring iron in the form of Fe2+. (wikipedia.org)
  • The enzyme, originally characterized from the bacterium Escherichia coli, can catalyze the transfer of CoA to L-carnitine, crotonobetaine and gamma-butyrobetaine. (expasy.org)
  • LeanGBB Gamma-Butyrobetaine(GBB) a precursor to l-carnitine which stimulates the production of endogenous l-carnitine. (arnoldsupplements.com)
  • Evidence indicates that the causal factor is a defect in the muscle carnitine transporter. (medscape.com)
  • Wilcken B, Wiley V, Sim KG, Carpenter K. Carnitine transporter defect diagnosed by newborn screening with electrospray tandem mass spectrometry. (medscape.com)
  • Vijay S, Patterson A, Olpin S, Henderson MJ, Clark S, Day C. Carnitine transporter defect: diagnosis in asymptomatic adult women following analysis of acylcarnitines in their newborn infants. (medscape.com)
  • This gene provides instructions for making a protein called OCTN2 that transports carnitine into cells. (medlineplus.gov)
  • L-carnitine is an amino acid derivative that transports fatty acids into your cells to be processed for energy. (healthline.com)
  • Carnitine transports long-chain acyl groups from fatty acids into the mitochondrial matrix , so that they can be broken down through beta-oxidation to acetate to obtain usable energy via the citric acid cycle . (wikidoc.org)
  • This makes L-carnitine a conditionally essential nutrient ( 2 ). (healthline.com)
  • At room temperature, pure carnitine is a whiteish powder, and a water-soluble zwitterion with relatively low toxicity. (wikipedia.org)
  • How much L Carnitine Powder should I take? (healthproductsforyou.com)
  • Novel OCTN2 mutations: no genotype-phenotype correlations: early carnitine therapy prevents cardiomyopathy. (medscape.com)
  • Given its key metabolic roles, carnitine is concentrated in tissues like skeletal and cardiac muscle that metabolize fatty acids as an energy source. (wikipedia.org)
  • With advancing age, carnitine levels decline in all of our tissues. (lifeextension.com)
  • Carnitine has shown to also be involved in the aging process as carnitine concentration in tissues declines with age and thereby reduces the integrity of the mitochondrial membrane. (professionalwhey.com.au)
  • L-carnitine may help increase mitochondrial function, which plays a key role in disease and healthy aging ( 1 , 10 , 11 ). (healthline.com)
  • A wealth of published data documents the ability of carnitine to improve insulin sensitivity, mitochondrial function, and cardiovascular health. (lifeextension.com)
  • 6-9 Loss of mitochondrial function in muscle tissue also leads to the familiar "flabby" body composition we associate with older people because it causes muscle atrophy and increased body fat mass. 10 From this list, you can see how essential carnitine is to your daily health. (lifeextension.com)
  • Fortunately, studies have proven that supplementing with carnitine can restore mitochondrial function to near-youthful levels. (lifeextension.com)
  • Carefully monitor adequate carnitine dose in primary and secondary carnitine deficiencies by evaluating plasma carnitine levels during follow-up visits. (medscape.com)
  • L-carnitine deficiencies can be a result of certain health conditions. (womanjunction.com)
  • Omnivorous subjects completed an L-carnitine challenge by eating a large amount of L-carnitine (an 8-oz sirloin steak with estimated 180 mg L-carnitine) as well as a capsule containing 250 mg of heavy isotope-labeled L-carnitine (d3-carnitine). (naturalmedicinejournal.com)
  • Low l-carnitine levels can be a result of a genetic disorder, medication, or a medical procedure. (womanjunction.com)
  • And it was formed in meat eaters, so maybe it's the meat and not the L-carnitine within the meat that that's promoting TMAO. (medscape.com)
  • In addition to the L-carnitine produced in your body, you can also obtain small amounts by eating animal products like meat or dairy products ( 2 ). (healthline.com)
  • The highest concentrations of carnitine are found in red meat and dairy products. (wikidoc.org)
  • Typically found in red meat, the amino acid, L-Carnitine, contributes to the release of energy from food. (naturesbest.co.uk)
  • Mingorance C, Rodríguez-Rodríguez R, Justo ML, Alvarez de Sotomayor M, Herrera MD, Critical update for the clinical use of L-carnitine analogs in cardiometabolic disorders. (aksci.com)
  • Longo N, Amat di San Filippo C, Pasquali M. Disorders of carnitine transport and the carnitine cycle. (medscape.com)
  • L-Carnitine reduced exercise-induced catabolism (muscle breakdown), reduced free-radical generation, and minimized muscle tissue disruption compared to the placebo. (bodybuilding.com)
  • In other words, L-Carnitine optimized a favorable anabolic recovery environment primed for dramatic muscle gains. (bodybuilding.com)
  • You've read the hard, clinical data proving that L-Carnitine is a potent, anabolic compound with powerful muscle-building properties. (bodybuilding.com)
  • L-Carnitine has also been shown to support muscle growth, weight management and liver health. (bayho.com)
  • However, logic tells us that if it allows one to remain active for a longer period of time, and longer periods of physical activity lead to leaner muscle mass and lower fat mass, it must follow that L-Carnitine plays an important part in weight reduction. (naturalremedies.org)
  • People with heart muscle damage from heart attacks or heart failure have especially low carnitine levels. (lifeextension.com)
  • By raising the level of muscle carnitine, you support the fat burning process, and because your body becomes more efficient at processing fuel, it will increase your energy levels. (supervits.com)
  • Elevating carnitine will also improve physical performance by burning more fat, sparing glycogen, clearing muscle lactate, and increasing anabolic hormone response. (supervits.com)
  • L-carnitine is also a great weapon for hardgainers looking to pack on muscle. (supervits.com)
  • It can be taken post-workout as L-carnitine is highly effective in easing off muscle soreness. (healthproductsforyou.com)
  • These patients experience progressive muscle atrophy that might cause impaired carnitine storage and might manifest as communication difficulties. (bvsalud.org)
  • Humans synthesize carnitine from the substrate TML (6-N-trimethyllysine), which is in turn derived from the methylation of the amino acid lysine. (wikipedia.org)
  • Tamai I. Pharmacological and pathophysiological roles of carnitine/organic cation transporters (OCTNs: SLC22A4, SLC22A5 and Slc22a21). (medscape.com)
  • This inactive form has been shown to reduce blood levels of carnitine and increase fat buildup, leading to liver inflammation and oxidative stress ( 4 ). (healthline.com)
  • The placebo group exhibited the beginning stages of non-alcoholic fatty liver disease and atherosclerosis, neither of which were evident in the carnitine group. (supervits.com)
  • L-carnitine is produced in the liver, kidney, and brain, and is then transferred to other parts of the body to support the breakdown. (womanjunction.com)
  • L-carnitine is beneficial to the overall health of the liver and heart [ 2 ], so making sure that your body produces enough is important to maintain the functional processes. (womanjunction.com)
  • L-carnitine can be used for several different conditions, such as lowering heart-related risks and keeping the heart and liver healthy . (womanjunction.com)
  • Germ-free mice underwent an L-carnitine challenge, which demonstrated no detectable plasma d3-TMAO production after ingestion of d3-carnitine. (naturalmedicinejournal.com)
  • Some studies have determined that taking l-carnitine after a heart attack decreases the chances of suffering another one later. (supervits.com)
  • SLC22A5 mutations can affect carnitine transport by impairing maturation of transporters to the plasma membrane. (medscape.com)
  • L-carnitine is an important metabolic mediator involved in fatty acid transport. (bvsalud.org)
  • Paul Wang, the senior vice president for medical research at AutismSpeaks, said he could not endorse adding significant amounts of carnitine to people's diets without clinical trials, but nonetheless said the science behind the idea is sound. (heraldnet.com)
  • Several clinical trials show that L-carnitine can be used along with conventional treatment for angina to reduce the needs for medicine and improve the ability of those with angina to exercise without chest pain or discomfort. (supervits.com)
  • Carnitine acyltransferase I undergoes allosteric inhibition as a result of malonyl-CoA , an intermediate in fatty acid biosynthesis, in order to prevent futile cycling between beta-oxidation and fatty acid synthesis. (wikidoc.org)
  • The first reaction of the carnitine shuttle is a two-step process catalyzed by a family of isozymes of acyl-CoA synthetase that are found in the outer mitochondrial membrane, where they promote the activation of fatty acids by forming a thioester bond between the fatty acid carboxyl group and the thiol group of coenzyme A to yield a fatty acyl-CoA. (wikipedia.org)
  • Carnitine binds acyl residues and helps in their elimination, decreasing the number of acyl residues conjugated with coenzyme A (CoA) and increasing the ratio between free and acylated CoA. (medscape.com)
  • Fatty acids must be activated before binding to the carnitine molecule to form acyl-carnitine . (wikidoc.org)
  • The acyl group on CoA can now be transferred to carnitine and the resulting acyl-carnitine transported into the mitochondrial matrix . (wikidoc.org)
  • While omnivores showed increases in TMAO and d3-TMAO postprandially, vegans showed nominal plasma and urine TMAO at baseline and had virtually no capacity to generate TMAO or d3-TMAO in response to the L-carnitine challenge. (naturalmedicinejournal.com)
  • Vegans also had a lower baseline plasma level of L-carnitine compared to omnivorous subjects. (naturalmedicinejournal.com)
  • Biologic effects of low carnitine levels may not be clinically significant until they reach less than 10-20% of normal. (medscape.com)
  • Carnitine levels decline with age. (lifeextension.com)
  • Raising your carnitine levels will fight this visceral fat gain because it increases fat burning, which has the effect of taking triglycerides and low-density lipoproteins out of the system so that they don't build up causing high cholesterol and atherosclerosis. (supervits.com)
  • This study looked at 27 advanced cancer patients with low serum l-carnitine levels and moderate to severe fatigue. (pallimed.org)
  • Previous reports have revealed that long-term tube feeding with a commercial product containing no or low levels of carnitine can lead to an altered mental state caused by hyperammonemia . (bvsalud.org)
  • His main enteral solution was a commercial product that contained low carnitine levels, and he sometimes received coffee and homemade products such as miso soup. (bvsalud.org)
  • Clinicians should be aware of this disorder and should consider periodically measuring carnitine levels, regardless of the patient 's serum ammonium levels. (bvsalud.org)
  • of the head shows fronto-temporal atrophy carnitine levels in urine were elevated and and bilateral subdural haemorrhage glutaryl-CoA dehydrogenase activity in cul- tured fibroblasts was low. (who.int)
  • We only use pure-grade Carnitine suitable for nutrition products in our formula. (naturesbest.co.uk)
  • Carnitine in neonatal nutrition. (medscape.com)
  • Considering these results, we conclude that L-carnitine has a protective effect against cardiac ischemic reperfusion injuries as a reduction of apoptotic cardiomyocytes. (vin.com)