HADHA
The enzyme converts medium- and long-chain 2-enoyl-CoA compounds into the following 3-ketoacyl-CoA when NAD is solely present, ... "Entrez Gene: Hydroxyacyl-CoA dehydrogenase/3-ketoacyl-CoA thiolase/enoyl-CoA hydratase (trifunctional protein), alpha subunit ... "hydroxyacyl-CoA dehydrogenase/3-ketoacyl-CoA thiolase/enoyl-CoA hydratase (trifunctional protein), alpha subunit". Cardiac ... Trifunctional enzyme subunit alpha, mitochondrial also known as hydroxyacyl-CoA dehydrogenase/3-ketoacyl-CoA thiolase/enoyl-CoA ...
Long-chain 3-hydroxyacyl-coenzyme A dehydrogenase deficiency
CoA) dehydrogenase, which is part of a protein complex known as mitochondrial trifunctional protein. Long-chain fatty acids ... Long-chain fatty acids or partially metabolized fatty acids may build up in tissues and damage the liver, heart, retina, and ... Long-chain 3-hydroxyacyl-coenzyme A dehydrogenase deficiency is a rare autosomal recessive fatty acid oxidation disorder that ... Medium chain acyl dehydrogenase deficiency Reference, Genetics Home. "LCHAD deficiency". Genetics Home Reference. Retrieved ...
Acute fatty liver of pregnancy
It is thought to be caused by a disordered metabolism of fatty acids by mitochondria in the fetus, caused by long-chain 3- ... hydroxyacyl-coenzyme A dehydrogenase deficiency. This leads to decreased metabolism of long chain fatty acids by the feto- ... "Disorders of mitochondrial fatty acyl-CoA beta-oxidation" (PDF). Journal of Inherited Metabolic Disease. 22 (4): 442-487. doi: ... Bellig LL (2004). "Maternal acute fatty liver of pregnancy and the associated risk for long-chain 3-hydroxyacyl-coenzyme a ...
HADHB
... long-chain enoyl-CoA hydratase, and long-chain thiolase. This deficiency can be classified into 3 main clinical phenotypes: ... "Entrez Gene: hydroxyacyl-Coenzyme A dehydrogenase/3-ketoacyl-Coenzyme A thiolase/enoyl-Coenzyme A hydratase (trifunctional ... Middleton B (1994). "The mitochondrial long-chain trifunctional enzyme: 2-enoyl-CoA hydratase, 3-hydroxyacyl-CoA dehydrogenase ... Trifunctional protein deficiency is characterized by decreased activity of long-chain 3-hydroxyacyl-CoA dehydrogenase (LCHAD), ...
Mitochondrial trifunctional protein deficiency
"Long-Chain Acyl CoA Dehydrogenase Deficiency: Background, Pathophysiology, Epidemiology". eMedicine. 24 March 2016. Retrieved ... "OMIM Entry - * 600890 - HYDROXYACYL-CoA DEHYDROGENASE/3-KETOACYL-CoA THIOLASE/ENOYL-CoA HYDRATASE, ALPHA SUBUNIT; HADHA". omim. ... long-chain enoyl-CoA hydratase, and long-chain thiolase activities. Trifunctional protein deficiency is characterized by ... Avoiding factors that might precipitate condition Glucose Low fat/high carbohydrate nutrition Long-chain acyl-CoA dehydrogenase ...
Mitochondrial trifunctional protein
"Long-Chain Acyl CoA Dehydrogenase Deficiency: eMedicine Pediatrics: Genetics and Metabolic Disease". Retrieved 2009-07-11. Wang ... CoA) hydratase, long-chain 3-hydroxy acyl-coenzyme A dehydrogenase and long-chain 3-ketoacyl CoA thiolase. Fatty acid beta- ...
Fatty-acid metabolism disorder
Very long-chain acyl-coenzyme A dehydrogenase deficiency (VLCAD deficiency) Long-chain 3-hydroxyacyl-coenzyme A dehydrogenase ... 4 Dienoyl-CoA Reductase Deficiency Electron Transfer Flavoprotein (ETF) Dehydrogenase Deficiency (GAII & MADD) 3-Hydroxy-3- ... Medium-chain acyl-coenzyme A dehydrogenase deficiency (MCAD deficiency) Short-chain acyl-coenzyme A dehydrogenase deficiency ( ... 3-hydroxyacyl-coenzyme A dehydrogenase deficiency (M/SCHAD deficiency) The term fatty acid oxidation disorder (FAOD) is ...
Beta oxidation
If the fatty acyl-CoA has a long chain, then the carnitine shuttle must be utilized: Acyl-CoA is transferred to the hydroxyl ... is not an appropriate substrate for acyl CoA dehydrogenase, or enoyl CoA hydratase: If the acyl CoA contains a cis-Δ3 bond, ... A long-chain fatty acid is dehydrogenated to create a trans double bond between C2 and C3. This is catalyzed by acyl CoA ... Long-chain-fatty-acid-CoA ligase catalyzes the reaction between a fatty acid with ATP to give a fatty acyl adenylate, plus ...
Inborn error of lipid metabolism
Very long-chain acyl-coenzyme A dehydrogenase deficiency (VLCAD) - Very long-chain acyl-coenzyme A dehydrogenase Long-chain 3- ... hydroxyacyl-coenzyme A dehydrogenase deficiency (LCHAD) - Long-chain 3-hydroxyacyl-coenzyme A Medium-chain acyl-coenzyme A ... 4 Dienoyl-CoA reductase deficiency - 2,4 Dienoyl-CoA reductase 3-hydroxy-3-methylglutaryl-CoA lyase deficiency - 3-hydroxy-3- ... Medium-chain acyl-coenzyme A dehydrogenase Short-chain acyl-coenzyme A dehydrogenase deficiency (SCAD) - Short-chain acyl- ...
List of causes of hypoglycemia
... hypoglycaemia Levomepromazine Liver cancer Liver glycogen synthase deficiency Long-chain hydroxyacyl-CoA dehydrogenase ... Trimethoprim Triple A syndrome Tumors Tyrosinaemia type 1 Urea cycle disorder Uremia Very-long-chain acyl-CoA dehydrogenase ... Reye syndrome Ritonavir Saquinavir Sepsis Septic shock Severe hepatitis Sheehan syndrome Short-chain acyl-CoA dehydrogenase ... deficiency Maple syrup urine disease Mcquarrie type infantile idiopathic hypoglycemia Medium chain acyl-CoA dehydrogenase ...
Acyl-CoA
These enzymes are better equipped to oxidize Acyl-CoA with long chains that the mitochondria cannot handle. Beta oxidation ... Beta oxidation of acyl-CoA occurs in four steps. 1. Acyl-CoA dehydrogenase catalyzes dehydrogenation of the acyl-CoA, creating ... The latter conversion is mediated by acyl-CoA synthase" acyl-P + HS-CoA → acyl-S-CoA + Pi + H+ Three types of acyl-CoA ... Acetyl-CoA Beta oxidation Coenzyme A Acyl CoA dehydrogenase Fatty acid metabolism Talley, Jacob T.; Mohiuddin, Shamim S. (2020 ...
List of disorders included in newborn screening programs
Short-chain hydroxy Acyl-CoA dehydrogenase deficiency (SCHAD) Long-chain acyl-CoA dehydrogenase deficiency (LCAD) Multiple acyl ... 1 in 75,000 Medium-chain acyl-CoA dehydrogenase deficiency (MCAD) > 1 in 25,000 Very-long-chain acyl-CoA dehydrogenase ... 1 in 100,000 Inborn errors of fatty acid metabolism Long-chain hydroxyacyl-CoA dehydrogenase deficiency (LCHAD) > ... butyric aciduria Isobutyryl-CoA dehydrogenase deficiency 2-Methylbutyryl-CoA dehydrogenase deficiency 3-Methylglutaconyl-CoA ...
Lipid metabolism
Long chain fatty acids (more than 14 carbon) need to be converted to fatty acyl-CoA in order to pass across the mitochondria ... The process of beta oxidation requires the following enzymes: acyl-CoA dehydrogenase, enoyl-CoA hydratase, 3-hydroxyacyl-CoA ... The diagram to the left shows how fatty acids are converted into acetyl-CoA. The overall net reaction, using palmitoyl-CoA (16: ... Short chain fatty acids can be absorbed in the stomach, while most absorption of fats occurs only in the small intestines. Once ...
Enoyl CoA isomerase
... and long-chain) of rat liver contain more than one enoyl Co-A isomerase. To further support the idea that short- and long-chain ... The dehydrogenase activity of enoyl-CoA occurs in the carboxyl-terminal. Upon further investigation of the CoA binding site on ... There are two divisions among the mitochondrial enoyl Co-A isomerase: short-chain and long-chain [4]. In an immunoblot, ... There was one enzyme which did not have binding specificity to this antibody: mitochondrial long-chain isomerase. Long-chain ...
Fatty acid metabolism
Once inside the cell, long-chain-fatty-acid-CoA ligase catalyzes the reaction between a fatty acid molecule with ATP (which is ... Dehydrogenation by acyl-CoA dehydrogenase, yielding 1 FADH2 Hydration by enoyl-CoA hydratase Dehydrogenation by 3-hydroxyacyl- ... Acetyl-CoA is formed into malonyl-CoA by acetyl-CoA carboxylase, at which point malonyl-CoA is destined to feed into the fatty ... Beta oxidation, in the mitochondrial matrix, then cuts the long carbon chains of the fatty acids (in the form of acyl-CoA ...
D-bifunctional protein deficiency
DBP is a stereospecific enzyme; hydratase domain forms only (R)-hydroxy-acyl-CoA intermediates from trans-2-enoyl-CoAs. D-BP is ... A non-functional D-BP protein results in the abnormal accumulation of long chain fatty acids and bile acid intermediates. The D ... the N-terminal short chain alcohol dehydrogenase reductase (SDR), central hydratase domain, and the C-terminal sterol carrier ... and branched chain acyl-CoA oxidase. Möller G, van Grunsven EG, Wanders RJ, Adamski J (January 2001). "Molecular basis of D- ...
List of OMIM disorder codes
SCARB2 Acyl-CoA dehydrogenase, long chain, deficiency of; 201460; ACADL Acyl-CoA dehydrogenase, medium chain, deficiency of; ... TGFBR2 Long QT syndrome 12; 612955; SNT1 Long QT syndrome 13; 613485; KCNJ5 Long QT syndrome-1; 192500; KCNQ1 Long QT syndrome- ... ACADM Acyl-CoA dehydrogenase, short chain, deficiency of; 201470; ACADS Adenocarcinoma of lung, response to tyrosine kinase ... SCN5A Long QT syndrome-4; 600919; ANK2 Long QT syndrome-7; 170390; KCNJ2 Long QT syndrome-9; 611818; CAV3 Lowe syndrome; 309000 ...
Short-chain acyl-CoA dehydrogenase
... medium-chain, and long-chain acyl-CoA dehydrogenases from rat liver mitochondria. Isolation of the holo- and apoenzymes and ... Short-chain acyl-CoA dehydrogenase (EC 1.3.8.1, butyryl-CoA dehydrogenase, butanoyl-CoA dehydrogenase, butyryl dehydrogenase, ... Acyl-CoA dehydrogenase Medium-chain acyl-CoA dehydrogenase Butyryl-CoA (also known as butanoyl-CoA) Mahler HR (January 1954). " ... butyryl coenzyme A dehydrogenase, short-chain acyl CoA dehydrogenase, short-chain acyl-coenzyme A dehydrogenase, 3-hydroxyacyl ...
List of EC numbers (EC 2)
... mycolipenoyl-CoA-2-(long-chain-fatty acyl)-trehalose mycolipenoyltransferase (*) EC 2.3.1.279: long-chain-acyl-CoA-trehalose ... isocitrate dehydrogenase (NADP+)] kinase EC 2.7.1.117: Now EC 2.7.11.18, myosin-light-chain kinase EC 2.7.1.118: ADP-thymidine ... cinnamoyl-CoA:phenyllactate CoA-transferase EC 2.8.3.18: succinyl-CoA:acetate CoA-transferase (*) EC 2.8.3.19: CoA:oxalate CoA- ... succinyl-CoA-L-malate CoA-transferase and EC 2.8.3.20, succinyl-CoA-Dcitramalate CoA-transferase EC 2.8.3.8: acetate CoA- ...
2,4 Dienoyl-CoA reductase
... hydroxyacyl-CoA dehydrogenase units". Journal of Molecular Biology. 358 (5): 1286-1295. doi:10.1016/j.jmb.2006.03.001. PMID ... at which this very long chain fatty acid is first partially oxidized by pDECR in the peroxisome. 2,4 Dienoyl-CoA thioester ... Additionally, at one end of the active site there is a flexible loop that provides sufficient room for long carbon chains. This ... Although pDCR can use 2,4-hexadienoyl CoA as a substrate, the affinities for short chain fatty acids are lower. Analysis of the ...
Enoyl-CoA hydratase
... of enoyl-CoA hydratase complexed with octanoyl-CoA reveals the structural adaptations required for binding of a long chain ... The α-carbon then grabs another proton, which completes the formation of the beta-hydroxy acyl-CoA. It is also known from ... whereas the dehydrogenase enzyme is found exclusively in the mitochondrion (Sabourin and Bieber 1981, 1983). Importantly, this ... This allows ECH to make an S stereoisomer from 2-trans-enoyl-CoA and an R stereoisomer from the 2-cis-enoyl-CoA. This is made ...
List of EC numbers (EC 1)
... medium-chain acyl-CoA dehydrogenase, EC 1.3.8.8, long-chain acyl-CoA dehydrogenase and EC 1.3.8.9, very-long-chain acyl-CoA ... medium-chain acyl-CoA dehydrogenase EC 1.3.8.8: long-chain acyl-CoA dehydrogenase EC 1.3.8.9: very-long-chain acyl-CoA ... long-chain-aldehyde dehydrogenase EC 1.2.1.49: 2-oxoaldehyde dehydrogenase (NADP+) EC 1.2.1.50: long-chain-fatty-acyl-CoA ... 2-methyl-branched-chain-enoyl-CoA reductase EC 1.3.99.13: Now EC 1.3.8.8, long-chain-acyl-CoA dehydrogenase EC 1.3.99.14: ...
Alcanivorax borkumensis
... a product that consists of a long chain of carbon atoms attached to hydrogen atoms. Whereas most organisms use sugars or amino ... By disrupting an acyl-coenzyme A (CoA) thioesterase gene, Sabirova and colleagues were able to mutate the organism to hyper- ... This sequential pathway first produces alcohols, then alcohol and aldehyde dehydrogenases, and ultimately aldehydes and fatty ... Hydroxyacyl-Coenzyme A-Specific Thioesterase Gene Causes Hyperproduction of Extracellular Polyhydroxyalkanoates by Alcanivorax ...
List of MeSH codes (D08)
... acyl-coa dehydrogenase, long-chain MeSH D08.811.682.660.150.200 - acyl-CoA oxidase MeSH D08.811.682.660.150.300 - butyryl-coa ... acyl-coa dehydrogenases MeSH D08.811.682.660.150.100 - acyl-coa dehydrogenase MeSH D08.811.682.660.150.150 - ... Glutaryl-CoA dehydrogenase MeSH D08.811.682.660.462 - isovaleryl-coa dehydrogenase MeSH D08.811.682.660.490 - 15- ... malate dehydrogenase MeSH D08.811.682.047.748 - malate dehydrogenase (nadp+) MeSH D08.811.682.047.892 - xanthine dehydrogenase ...
HSD17B4
"Peroxisomal D-hydroxyacyl-CoA dehydrogenase deficiency: resolution of the enzyme defect and its molecular basis in bifunctional ... medium-chain enoyl-CoA hydratase is D-3-hydroxyacyl-CoA dehydratase". Journal of Biochemistry. 120 (3): 624-32. doi:10.1093/ ... v t e (Articles with short description, Short description matches Wikidata, Genes on human chromosome 5, CS1: long volume value ... short-chain dehydrogenase/reductase and related enzymes) nomenclature initiative". Chemico-Biological Interactions. 178 (1-3): ...
Congenital hyperinsulinism
Examples include changes in the genes that make the enzymes hydroxyacyl-CoA dehydrogenase gene (SCHAD-CHI) and glucokinase (GCK ... Because of its long half-life it may take up to 5 days to achieve a full therapeutic effect. An important next step is the ... Zhang, Wei; Sang, Yan-Mei (4 November 2021). "Genetic pathogenesis, diagnosis, and treatment of short-chain 3-hydroxyacyl- ... More commonly, many on diazoxide develop excess body hair or a change in facial features over a long time. Children with focal ...