This enzyme complex metabolizes long-chain fatty acids, and the long-chain 3-hydroxy acyl-coenzyme A dehydrogenase activity is specific for compounds of C12-C16 chain length. (medscape.com)
Some patients who are deficient in all 3 enzymatic activities of the protein have been described, although most have an isolated long-chain 3-hydroxy acyl-coenzyme A dehydrogenase deficiency, which results in the inability to metabolize long-chain fatty acids. (medscape.com)
Thus, the clinical features may result from either toxicity due to long-chain acyl-CoA esters that cause cardiomyopathy and cardiac arrhythmias or from a block in long-chain fatty acid oxidation that leads to an inability to synthesize ketone bodies and/or adenosine triphosphate from long-chain fatty acids. (medscape.com)
Adrenoleukodystrophy protein (ALDP) is responsible for the transport of very-long-chain fatty acids (VLCFAs) and corresponding CoA-esters across the peroxisomal membrane. (elifesciences.org)
The adrenoleukodystrophy protein (ALDP) or ABCD1 is an ABC transporter that participates in the transport of free very long-chain fatty acids and their CoA esters across the peroxisomal membrane. (elifesciences.org)
Those with a longer chain use different transport systems in the membrane enabling their facilitated diffusion - for example FATP (fatty acid transport protein) or FAT/CD36 (fatty acid translocase). (wikilectures.eu)
Fatty acid activation occurs in the cytosol, on the outer mitochondrial membrane immediately after their entry into the cell. (wikilectures.eu)
Moiety3
That is, α -TOS comprises a hydrophobic phytyl chain (hydrophobic domain) and the chargeable succinyl group (functional domain), separated by the bulky tocopheryl moiety (signalling domain) ( Neuzil, 2002 ). (nature.com)
The eicosapentaenoic acid moiety is derived from fish oils, liver and kidney, while the docosapentaenoic acid moiety is derived from fish oils. (hmdb.ca)
These are glycerophosphoglycerophosphates in which two fatty acids are bonded to the 1-glycerol moiety through ester linkages. (ymdb.ca)
Amino acids2
Propionic acidemia (PA) and isolated methylmalonic acidemia (MMA) are disorders affecting the catabolic pathway of the branched-chain amino acids (BCAA) L-isoleucine and L-valine, and the amino acids L-threonine and L-methionine. (biomedcentral.com)
The apoptotic cell cargo contains amino acids, nucleotides, fatty acids, and cholesterol that function as metabolites and signaling molecules to bring about this re-programming. (biomed.news)
Oxidation12
Common industrial routes include hydrogenation of maleic acid, oxidation of 1,4-butanediol, and carbonylation of ethylene glycol. (wikipedia.org)
Schematic demonstrating mitochondrial fatty acid beta-oxidation and effects of long-chain acyl CoA dehydrogenase deficiency (LCHAD) deficiency. (medscape.com)
However, this effect may be achieved at the cost of fatty acid infiltration and of toxic effects of β-oxidation intermediates on organ functions. (medscape.com)
The fatty acid oxidation defect results in adverse effects on numerous organ systems, including the CNS, secondary to the hypoketotic hypoglycemia. (medscape.com)
Although the dicarboxylic acids do not occur in appreciable amounts as components of animal or vegetal lipids, they are in general important metabolic products of fatty acids since they originate from them by oxidation . (gerli.com)
by oxidation of castor oil with nitric acid (splitting of the carbon chain close to the OH group). (gerli.com)
A graphic chart describing the biosynthesis of adipic acid via omega oxidation may be found on the BioCarta web site. (gerli.com)
Suberic acid (n=6) : it was firstly produced by nitric acid oxidation of cork (Latin suber ) material and then from castor oil ( Tilley TG, Ann 1841, 39, 160 ). (gerli.com)
The oxidation of ricinoleic acid produces, by splitting at the level of the double bond and at the level of the OH group, at the same time, suberic acid (octanedioic acid) and the next homologue azelaic acid. (gerli.com)
Its name stems from the action of nitric acid ( azote , nitrogen, or azotic , nitric) oxidation of oleic or elaidic acid. (gerli.com)
Individual types of fatty acid oxidation are indicated by Greek letters, which determine the carbon atom on which the reactions take place. (wikilectures.eu)
Metabolites1
1985. Mutagenicity evaluation of phthalic acid esters and metabolites in Salmonella typhimurium cultures. (cdc.gov)
Fatty acid3
We found that the antiviral activity of LCFA-CoA relied on the long-chain saturated fatty acid and the CoA group, and was enhanced when combined with pegylated-interferon or DAAs. (bvsalud.org)
The activation of the fatty acid actually passes off in two stages. (wikilectures.eu)
Disruption of adipocyte de novo lipogenesis (DNL) by deletion of fatty acid synthase (FASN) in mice induces browning in inguinal white adipose tissue (iWAT). (biomed.news)
Phosphatidic acid2
In one route, choline is activated first by phosphorylation and then by coupling to CDP prior to attachment to phosphatidic acid. (hmdb.ca)
In E. coli glycerophospholipid metabolism, The biosynthesis of CDP-diacylglycerol (CDP-DG) involves condensation of phosphatidic acid (PA) and cytidine triphosphate, with elimination of pyrophosphate, catalysed by the enzyme CDP-diacylglycerol synthase. (ymdb.ca)
Acyl2
Patients with long-chain 3-hydroxy acyl-coenzyme A dehydrogenase deficiency may develop a profound CNS deficiency of docosahexanoic acid ethyl ester (DHA), 22:6n-3. (medscape.com)
It was revealed that Cys-Dan, which has both a thiol and an amino group, was the most effective in forming stable adducts containing an amide bond after intramolecular acyl migration. (bvsalud.org)
Metabolic3
In living organisms, succinic acid takes the form of an anion, succinate, which has multiple biological roles as a metabolic intermediate being converted into fumarate by the enzyme succinate dehydrogenase in complex 2 of the electron transport chain which is involved in making ATP, and as a signaling molecule reflecting the cellular metabolic state. (wikipedia.org)
citation needed] Succinate is a key intermediate in the tricarboxylic acid cycle, a primary metabolic pathway used to produce chemical energy in the presence of O2. (wikipedia.org)
Based on this, we suggest to measure plasma 3-hydroxyisovaleric acid and urinary ketones or 3-hydroxybutyric acid for the biochemical follow-up of a patient's metabolic stability. (biomedcentral.com)
Succinyl1
As a radical group it is called a succinyl (/ˈsʌksɪnəl/) group. (wikipedia.org)
Organic1
Dicarboxylic acids are suitable substrates for preparation of organic acids for the pharmaceutical and food industries. (gerli.com)
Intermediates2
The simplest of these intermediates is oxalic acid (n=0), the others are malonic (n=1), succinic (n=2) and glutaric (n=3) acids. (gerli.com)
We found that known diagnostic biomarkers were not significantly further increased, while intermediates of the branched-chain amino acid (BCAA) degradation pathway were significantly increased during AMD. (biomedcentral.com)
Succinic13
Succinic acid (/səkˈsɪnɪk/) is a dicarboxylic acid with the chemical formula (CH2)2(CO2H)2. (wikipedia.org)
Succinic acid is marketed as food additive E363. (wikipedia.org)
Succinic acid is a white, odorless solid with a highly acidic taste. (wikipedia.org)
As a diprotic acid, succinic acid undergoes two successive deprotonation reactions: (CH2)2(CO2H)2 → (CH2)2(CO2H)(CO2)− + H+ (CH2)2(CO2H)(CO2)− → (CH2)2(CO2)22− + H+ The pKa of these processes are 4.3 and 5.6, respectively. (wikipedia.org)
Historically, succinic acid was obtained from amber by distillation and has thus been known as spirit of amber. (wikipedia.org)
Succinic acid can be dehydrogenated to fumaric acid or be converted to diesters, such as diethylsuccinate (CH2CO2CH2CH3)2. (wikipedia.org)
citation needed] As a food additive and dietary supplement, succinic acid is generally recognized as safe by the U.S. Food and Drug Administration. (wikipedia.org)
Succinic acid is used primarily as an acidity regulator in the food and beverage industry. (wikipedia.org)
Mitochondrial1
Mitochondria play a critical role in differentiation events in cellular systems, thus we hypothesized that mitochondrial metabolism played a central role in trophoblast differentiation. (biomed.news)
Occur1
One manifestation of negative selection in the globins is that minimal 3-base type amino acid replacements occur less frequently than would be expected if base replacements had occurred and were accepted at random. (pharmaceuticalintelligence.com)
Metabolism1
Short-chain dicarboxylic acids are of great importance in the general metabolism and up to n=3 they cannot be considered as lipids since their water solubility is important. (gerli.com)
Peroxisome1
1987. Peroxisome induction studies on seven phthalate esters. (cdc.gov)
Fatty acids represent a rich source of energy for gluconeogenesis , but glucose is not formed from their carbon atoms (with the exception of fatty acids with an odd number of C). Acetyl-CoA cannot be converted to either pyruvate or oxaloacetate - both carbons are split off as CO 2 during the Krebs cycle . (wikilectures.eu)
Azelaic acid is used, as simple esters or branched-chain esters) in the manufacture of plasticizers (for vinyl chloride resins, rubber), lubricants and greases. (gerli.com)
Adipic acid (n=4) : Despite its name (in Latin adipis is fat), this acid (hexanedioic acid) is not a normal constituent of natural lipids but is a product of oxidative rancidity (lipid peroxidation). (gerli.com)
Sebacic acid (n=8) : decanedioic acid was named by Thenard LJ (1802) from the Latin sebaceus (tallow candle) or sebum (tallow) in reference to its use in the manufacture of candles. (gerli.com)
Greek1
Pimelic acid (n=5) : this acid (heptanedioic acid), from the Greek pimelh (pimele fat), as adipic acid, was isolated from oxidized fats. (gerli.com)
Cells1
Mechanistic studies have revealed that MCCA-KD cells had dysfunctional mitochondria with decreased Bax and Bad levels and increased Bcl-xl and Mcl-1 levels. (bvsalud.org)
Transports1
The released fatty acids bind to serum albumin , which transports them to their destination (e.g. the liver). (wikilectures.eu)
Chemicals1
The term covers an entire group of chemicals, and studies have shown that they can affect the human endocrine system, which includes human reproductive organs. (debuglies.com)
State1
Citrate was preferentially exported from mitochondria in the undifferentiated state but was retained to a larger extent within mitochondria upon differentiation. (biomed.news)
Food1
The regulation prohibits the addition of any other antioxidant than lecithin, ascorbic Acid, and tocopherol to any food. (microbiologynote.com)
Patients2
Patients from all ethnic groups have been reported. (medscape.com)
We revealed that plasma leucine and phenylalanine, and urinary pyruvic acid were increased during AMD in PA patients. (biomedcentral.com)
Material1
The interactions of phthalate esters with suspended particulate material in fresh and marine waters. (cdc.gov)
Found1
The other lipid members of the group found in natural products or from synthesis have a 'n' value from 4 up to 21. (gerli.com)
Skin1
Acne is a common but serious skin disease, which affects approximately 80% adolescents and young adults in 11-30 age group. (hindawi.com)