Carnitine
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, ...
Carnitine decarboxylase
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. ...
Carnitine biosynthesis
... 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. ...
Carnitine dehydratase
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 ...
Carnitine 3-dehydrogenase
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 ...
Carnitine O-octanoyltransferase
... (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 palmitoyltransferase II
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 ...
Carnitine O-palmitoyltransferase
... (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 ...
Carnitine-acylcarnitine translocase
... (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 O-acetyltransferase
... 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 ...
Carnitine palmitoyltransferase I
... (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 ...
Systemic primary carnitine deficiency
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 ...
Carnitine palmitoyltransferase I deficiency
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 palmitoyltransferase II deficiency
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 ...
Glycine propionyl-L-carnitine
... (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 ...
S)-carnitine 3-dehydrogenase
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". ...
Carnitine-acylcarnitine translocase deficiency
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 ...
Meldonium
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 ...
Trimethyllysine dioxygenase
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 ...
3-Dehydrocarnitine
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. ...
B vitamins
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 ...
Transferase
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 ...
Lysine
... 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. ...
Gluten-sensitive enteropathy-associated conditions
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 ...
Trimethylaminuria
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 ...
List of OMIM disorder codes
PRKAR1A Carnitine deficiency, systemic primary; 212140; SLC22A5 Carotid intimal medial thickness 1; 609338; PPARG Carpal tunnel ...
Beta-ketothiolase deficiency
... 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 ...
Hyperammonemia
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 ...
Pseudoathletic appearance
Sinclair, Christopher; Gilchrist, James M.; Hennessey, James V.; Kandula, Manju (September 2005). "Muscle carnitine in hypo- ...
Dilated cardiomyopathy
... 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 ...