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- ...
Rhabdomyolysis
... deficiency of subtypes of acyl CoA dehydrogenase (LCAD, SCAD, MCAD, VLCAD, 3-hydroxyacyl-coenzyme A dehydrogenase deficiency), ... Elevated concentrations of the enzyme lactate dehydrogenase (LDH) may be detected. Other markers of muscle damage, such as ... glucose-6-phosphate dehydrogenase deficiency, myoadenylate deaminase deficiency and muscular dystrophies Damage to skeletal ... thiolase deficiency Mitochondrial myopathies: deficiency of succinate dehydrogenase, cytochrome c oxidase and coenzyme Q10 ...
List of MeSH codes (D08)
... acyl-coa dehydrogenases MeSH D08.811.682.660.150.100 - acyl-coa dehydrogenase MeSH D08.811.682.660.150.150 - acyl-coa ... Glutaryl-CoA dehydrogenase MeSH D08.811.682.660.462 - isovaleryl-coa dehydrogenase MeSH D08.811.682.660.490 - 15- ... acyl-CoA oxidase MeSH D08.811.682.660.150.300 - butyryl-coa dehydrogenase MeSH D08.811.682.660.200 - cholestenone 5alpha- ... malate dehydrogenase MeSH D08.811.682.047.748 - malate dehydrogenase (nadp+) MeSH D08.811.682.047.892 - xanthine dehydrogenase ...
HSD17B12
Estradiol 17-beta-dehydrogenase 12 is an enzyme that in humans is encoded by the HSD17B12 gene. The enzyme 17-beta ... Luu-The V, Tremblay P, Labrie F (2006). "Characterization of type 12 17beta-hydroxysteroid dehydrogenase, an isoform of type 3 ... "Entrez Gene: HSD17B12 hydroxysteroid (17-beta) dehydrogenase 12". Maruyama K, Sugano S (1994). "Oligo-capping: a simple method ... 2006). "Systemic distribution and tissue localizations of human 17beta-hydroxysteroid dehydrogenase type 12". J. Steroid ...
Crotonyl-CoA reductase
... (EC 1.3.1.86, butyryl-CoA dehydrogenase, butyryl dehydrogenase, unsaturated acyl-CoA reductase, ethylene ... short-chain acyl CoA dehydrogenase, short-chain acyl-coenzyme A dehydrogenase, 3-hydroxyacyl CoA reductase, butanoyl-CoA:( ... Crotonyl-CoA+reductase at the U.S. National Library of Medicine Medical Subject Headings (MeSH) Portal: Biology (Articles with ... Wallace KK, Bao ZY, Dai H, Digate R, Schuler G, Speedie MK, Reynolds KA (November 1995). "Purification of crotonyl-CoA ...
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 ... butyric aciduria Isobutyryl-CoA dehydrogenase deficiency 2-Methylbutyryl-CoA dehydrogenase deficiency 3-Methylglutaconyl-CoA ... 1 in 100,000 Inborn errors of fatty acid metabolism Long-chain hydroxyacyl-CoA dehydrogenase deficiency (LCHAD) > ...
Fatty-acid metabolism disorder
4 Dienoyl-CoA Reductase Deficiency Electron Transfer Flavoprotein (ETF) Dehydrogenase Deficiency (GAII & MADD) 3-Hydroxy-3- ... methylglutaryl-CoA lyase deficiency (HMG deficiency) Very long-chain acyl-coenzyme A dehydrogenase deficiency (VLCAD deficiency ... 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
... is not an appropriate substrate for acyl CoA dehydrogenase, or enoyl CoA hydratase: If the acyl CoA contains a cis-Δ3 bond, ... This is catalyzed by acyl CoA dehydrogenase to produce trans-delta 2-enoyl CoA. It uses FAD as an electron acceptor and it is ... The overall reaction for one cycle of beta oxidation is: Cn-acyl-CoA + FAD + NAD+ + H 2O + CoA → Cn-2-acyl-CoA + FADH 2 + NADH ... The final cycle produces two separate acetyl CoAs, instead of one acyl CoA and one acetyl CoA. For every cycle, the Acyl CoA ...
List of diseases (0-9)
11 beta hydroxysteroid dehydrogenase type 2 deficiency 17 alpha hydroxylase deficiency 17 beta hydroxysteroide dehydrogenase ... 2-Methylacetoacetyl CoA thiolase deficiency, rare (NIH) 2,8 dihydroxy-adenine urolithiasis 21 hydroxylase deficiency 22q11.2 ... coa hydratase deficiency 3-hydroxy 3-methyl glutaryl-coa lyase deficiency 3-hydroxyacyl-coa dehydrogenase deficiency 3-M ... deficiency 17-beta-hydroxysteroid dehydrogenase deficiency, rare (NIH) 17q21.31 microdeletion syndrome 18-Hydroxylase ...
Crotonase family
... a Pseudomonas enzyme which catalyses the conversion of 4-chlorobenzoate-CoA to 4-hydroxybenzoate-CoA. Dienoyl-CoA isomerase, ... 5-cis-dienoyl-CoA to 2-trans,4-trans-dienoyl-CoA. Naphthoate synthase (MenB, or DHNA synthetase; EC 4.1.3.36), a bacterial ... The CoA thioester derivatives bind in a characteristic hooked shape and a conserved tunnel binds the pantetheine group of CoA, ... an enoyl-CoA hydratase and a CoA-transferase, in the hydration of crotonobetaine to L-carnitine by Escherichia coli". ...
3-hydroxybutyryl-CoA epimerase
Wakil SJ (1955). "D(-)beta-Hydroxybutyryl CoA dehydrogenase". Biochim. Biophys. Acta. 18 (2): 314-315. doi:10.1016/0006-3002(55 ... Stern JR, del Campillo A, Lehninger AL (1955). "Enzymatic racemization of beta-hydroxybutyryl-S-CoA and the stereospecificity ... In enzymology, a 3-hydroxybutyryl-CoA epimerase (EC 5.1.2.3) is an enzyme that catalyzes the chemical reaction (S)-3- ... hydroxybutanoyl-CoA ⇌ {\displaystyle \rightleftharpoons } (R)-3-hydroxybutanoyl-CoA Hence, this enzyme has one substrate, (S)-3 ...
Chromosome 3
... encoding protein 2-hydroxyacyl-CoA lyase 1 HEMK1: encoding protein HemK methyltransferase family member 1 HIGD1A: HIG1 domain ... encoding enzyme NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 3 NKTR: NK-tumor recognition protein NPRL2: ... Chromosome 3 spans 201 million base pairs (the building material of DNA) and represents about 6.5 percent of the total DNA in ... Chromosome 3 is one of the 23 pairs of chromosomes in humans. People normally have two copies of this chromosome. ...
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 ... DBP deficiency can be divided into three types: type I, characterized by a deficiency in both the hydratase and dehydrogenase ... van Grunsven EG, Mooijer PA, Aubourg P, Wanders RJ (August 1999). "Enoyl-CoA hydratase deficiency: identification of a new type ... This breaks the hydrophobic interactions necessary for proper substrate binding with CoA esters. The most common clinical ...
List of EC numbers (EC 2)
... cinnamoyl-CoA:phenyllactate CoA-transferase EC 2.8.3.18: succinyl-CoA:acetate CoA-transferase (*) EC 2.8.3.19: CoA:oxalate CoA- ... 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 ... succinyl-CoA-L-malate CoA-transferase and EC 2.8.3.20, succinyl-CoA-Dcitramalate CoA-transferase EC 2.8.3.8: acetate CoA- ... L-carnitine CoA-transferase (*) EC 2.8.3.22: succinyl-CoA-L-malate CoA-transferase (*) EC 2.8.3.23: caffeate CoA-transferase ...
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 ... DECR is the second such enzyme (the others being enoyl CoA isomerase and dienoyl CoA isomerase) and is the rate limiting step ... A further distinction is E. Coli DECR produces the final 2-trans-enoyl-CoA without the need for Enoyl CoA Isomerase. The active ... DECR is capable of reducing both 2-trans,4-cis-dienoyl-CoA and 2-trans,4-trans-dienoyl-CoA thioesters with equal efficiency. ...
Spring peeper
These types of males utilize citrate synthase and β-hydroxyacyl CoA dehydrogenase in their muscles at greater levels. Males ... They have a body length between less than 25 mm (0.98 in) to 38 mm (1.5 in) and a mass between 3 and 5 g (0.11 and 0.18 oz). ... 3 (14): 4621-4630. doi:10.1002/ece3.851. PMC 3867898. PMID 24363891. Harding, James H. (1997). Amphibians and reptiles of the ... 1996 (3): 599-605. doi:10.2307/1447523. JSTOR 1447523. "Pseudacris crucifer". The Regents of the University of Michigan and its ...
Congenital hyperinsulinism
Examples include changes in the genes that make the enzymes hydroxyacyl-CoA dehydrogenase gene (SCHAD-CHI) and glucokinase (GCK ... Another common genetic cause of HI results from changes in the gene for the enzyme glutamate dehydrogenase (GDH). This genetic ... Shortly thereafter, mutations in glucokinase (GCK) and glutamate dehydrogenase (GLUD1) were also identified to cause HI. The ... "Hyperinsulinism and Hyperammonemia in Infants with Regulatory Mutations of the Glutamate Dehydrogenase Gene". New England ...
Citric acid cycle
Acetyl-coA inhibits pyruvate dehydrogenase, while succinyl-CoA inhibits alpha-ketoglutarate dehydrogenase and citrate synthase ... Lastly, beta-hydroxyacyl-CoA is oxidized to beta-ketoacyl-CoA while NAD+ is reduced to NADH, which follows the same process as ... Following, trans-Enoyl-CoA is hydrated across the double bond to beta-hydroxyacyl-CoA, just like fumarate is hydrated to malate ... inhibits pyruvate dehydrogenase, isocitrate dehydrogenase, α-ketoglutarate dehydrogenase, and also citrate synthase. ...
Enoyl-CoA hydratase
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 ... Following this pathway, HMB in the cytosol is first converted to cytosolic β-hydroxy-β-methylglutaryl-CoA (HMG-CoA), which can ...
Alcanivorax borkumensis
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 ... 18 (3): 257-266. CiteSeerX 10.1.1.475.3300. doi:10.1016/j.copbio.2007.04.006. PMID 17493798. Sabirova, Julia S.; Ferrer, Manuel ...