An NAD+ dependent enzyme that catalyzes the oxidation 3-methyl-2-oxobutanoate to 2-methylpropanoyl-CoA. It plays a role in the degradation of VALINE; LEUCINE; and ISOLEUCINE.
The addition of an organic acid radical into a molecule.
An enzyme that plays a role in the VALINE; LEUCINE; and ISOLEUCINE catabolic pathways by catalyzing the oxidation of 2-methyl-3-oxopropanate to propanoyl-CoA using NAD+ as a coenzyme. Methylmalonate semialdehyde dehydrogenase deficiency is characterized by elevated BETA-ALANINE and 3-hydropropionic acid.
A plant genus of the family ROSACEAE known for the edible fruit.
An enzyme that oxidizes an aldehyde in the presence of NAD+ and water to an acid and NADH. This enzyme was formerly classified as EC 1.1.1.70.
Organic compounds containing a carbonyl group in the form -CHO.
A plant genus in the family ROSACEAE and order Rosales. This should not be confused with the genus RHODIOLA which is sometimes called roseroot.
Oxidoreductases that are specific for ALDEHYDES.
A coenzyme composed of ribosylnicotinamide 5'-diphosphate coupled to adenosine 5'-phosphate by pyrophosphate linkage. It is found widely in nature and is involved in numerous enzymatic reactions in which it serves as an electron carrier by being alternately oxidized (NAD+) and reduced (NADH). (Dorland, 27th ed)
A metalloflavoprotein enzyme involved the metabolism of VITAMIN A, this enzyme catalyzes the oxidation of RETINAL to RETINOIC ACID, using both NAD+ and FAD coenzymes. It also acts on both the 11-trans- and 13-cis-forms of RETINAL.
An enzyme that converts brain gamma-aminobutyric acid (GAMMA-AMINOBUTYRIC ACID) into succinate semialdehyde, which can be converted to succinic acid and enter the citric acid cycle. It also acts on beta-alanine. EC 2.6.1.19.
The terms, expressions, designations, or symbols used in a particular science, discipline, or specialized subject area.
A characteristic feature of enzyme activity in relation to the kind of substrate on which the enzyme or catalytic molecule reacts.
The rate dynamics in chemical or physical systems.
Descriptions of specific amino acid, carbohydrate, or nucleotide sequences which have appeared in the published literature and/or are deposited in and maintained by databanks such as GENBANK, European Molecular Biology Laboratory (EMBL), National Biomedical Research Foundation (NBRF), or other sequence repositories.
Heterocyclic compounds in which an oxygen is attached to a cyclic nitrogen.
An autosomal recessive inherited disorder with multiple forms of phenotypic expression, caused by a defect in the oxidative decarboxylation of branched-chain amino acids (AMINO ACIDS, BRANCHED-CHAIN). These metabolites accumulate in body fluids and render a "maple syrup" odor. The disease is divided into classic, intermediate, intermittent, and thiamine responsive subtypes. The classic form presents in the first week of life with ketoacidosis, hypoglycemia, emesis, neonatal seizures, and hypertonia. The intermediate and intermittent forms present in childhood or later with acute episodes of ataxia and vomiting. (From Adams et al., Principles of Neurology, 6th ed, p936)
A ketone oxidoreductase that catalyzes the overall conversion of alpha-keto acids to ACYL-CoA and CO2. The enzyme requires THIAMINE DIPHOSPHATE as a cofactor. Defects in genes that code for subunits of the enzyme are a cause of MAPLE SYRUP URINE DISEASE. The enzyme was formerly classified as EC 1.2.4.3.
Oxidoreductases that are specific for KETONES.
3-((4-Amino-2-methyl-5-pyrimidinyl)methyl)-5-(2- hydroxyethyl)-4-methylthiazolium chloride.
Amino acids which have a branched carbon chain.
The coenzyme form of Vitamin B1 present in many animal tissues. It is a required intermediate in the PYRUVATE DEHYDROGENASE COMPLEX and the KETOGLUTARATE DEHYDROGENASE COMPLEX.
The dynamic collection of metabolites which represent a cell's or organism's net metabolic response to current conditions.
The systematic identification and quantitation of all the metabolic products of a cell, tissue, organ, or organism under varying conditions. The METABOLOME of a cell or organism is a dynamic collection of metabolites which represent its net response to current conditions.
The largest country in North America, comprising 10 provinces and three territories. Its capital is Ottawa.
A family of gram-negative bacteria, in the phylum FIRMICUTES.
A province of Canada on the Pacific coast. Its capital is Victoria. The name given in 1858 derives from the Columbia River which was named by the American captain Robert Gray for his ship Columbia which in turn was named for Columbus. (From Webster's New Geographical Dictionary, 1988, p178 & Room, Brewer's Dictionary of Names, 1992, p81-2)
The systematic study of the complete DNA sequences (GENOME) of organisms.
A province of western Canada, lying between the provinces of British Columbia and Saskatchewan. Its capital is Edmonton. It was named in honor of Princess Louise Caroline Alberta, the fourth daughter of Queen Victoria. (From Webster's New Geographical Dictionary, 1988, p26 & Room, Brewer's Dictionary of Names, 1992, p12)
... acylating) (EC 1.2.1.25) is an enzyme that catalyzes the chemical reaction 3-methyl-2-oxobutanoate + CoA + NAD+ ⇌ {\ ... and alpha-ketoisovalerate dehydrogenase. This enzyme participates in valine, leucine and isoleucine degradation. Namba Y, ... and nicotinamide adenine dinucleotide-dependent branched chain alpha-keto acid dehydrogenase. I. Purification and properties of ... The systematic name of this enzyme class is 3-methyl-2-oxobutanoate:NAD+ 2-oxidoreductase (CoA-methyl-propanoylating). Other ...
... methylmalonate-semialdehyde dehydrogenase (acylating) MeSH D08.811.682.657.163.835 - retinal dehydrogenase MeSH D08.811.682.657 ... malate dehydrogenase MeSH D08.811.682.047.748 - malate dehydrogenase (nadp+) MeSH D08.811.682.047.892 - xanthine dehydrogenase ... acetoin dehydrogenase MeSH D08.811.682.047.070 - alcohol dehydrogenase MeSH D08.811.682.047.150 - carbohydrate dehydrogenases ... acylating) MeSH D08.811.682.657.350.875 - pyruvate dehydrogenase (lipoamide) MeSH D08.811.682.657.350.937 - pyruvate oxidase ...
... glutamate-5-semialdehyde dehydrogenase EC 1.2.1.42: hexadecanal dehydrogenase (acylating) EC 1.2.1.43: formate dehydrogenase ( ... EC 1.1.1.1: alcohol dehydrogenase EC 1.1.1.2: alcohol dehydrogenase (NADP+) EC 1.1.1.3: homoserine dehydrogenase EC 1.1.1.4: (R ... acylating) EC 1.2.1.28: benzaldehyde dehydrogenase (NAD+) EC 1.2.1.29: aryl-aldehyde dehydrogenase EC 1.2.1.30: aryl-aldehyde ... acylating) EC 1.2.1.18: malonate-semialdehyde dehydrogenase (acetylating) EC 1.2.1.19: aminobutyraldehyde dehydrogenase EC 1.2. ...
... acylating) (EC 1.2.1.25) is an enzyme that catalyzes the chemical reaction 3-methyl-2-oxobutanoate + CoA + NAD+ ⇌ {\ ... and alpha-ketoisovalerate dehydrogenase. This enzyme participates in valine, leucine and isoleucine degradation. Namba Y, ... and nicotinamide adenine dinucleotide-dependent branched chain alpha-keto acid dehydrogenase. I. Purification and properties of ... The systematic name of this enzyme class is 3-methyl-2-oxobutanoate:NAD+ 2-oxidoreductase (CoA-methyl-propanoylating). Other ...
... acylating). An NAD+ dependent enzyme that catalyzes the oxidation 3-methyl-2-oxobutanoate to 2-methylpropanoyl-CoA. It plays a ... 2-Isopropylmalate synthase (IPMS) catalyzes the first step of leucine biosynthesis and is regulated via feedback inhibition by ... Alanine/glycine and rice powder containing ORS demonstrated some reduction in the stool output [2-5]. However, none of the ... 2. Reducing the concentration of glucose and sodium and thus osmolarity of ORS solution. ...
1.2.1.58 phenylglyoxylate dehydrogenase (acylating) 1.2.1.59 glyceraldehyde-3-phosphate dehydrogenase (NAD(P)+) ( ... 101298824 aldehyde dehydrogenase family 7 member A1 K00128 ALDH; aldehyde dehydrogenase (NAD+) [EC:1.2.1.3] K00128 ALDH; ... 101311533 aldehyde dehydrogenase family 3 member H1-like 101313059 aldehyde dehydrogenase family 3 member H1-like 101310892 ... 1.2.1.3 aldehyde dehydrogenase (NAD+) 101298221 aldehyde dehydrogenase family 2 member B4 101311183 aldehyde dehydrogenase ...
... acylating) Current Synonym true false 3721199012 2-oxoisovalerate dehydrogenase (acylating) Current Synonym true false ... 2-oxoisovalerate dehydrogenase (acylating) (substance). Code System Preferred Concept Name. 2-oxoisovalerate dehydrogenase ( ...
Acylating) * Pyruvate Dehydrogenase (Lipoamide) [D08.811.682.657.350.875] Pyruvate Dehydrogenase (Lipoamide) ... Dehydrogenase, alpha-Ketoisovalerate. alpha Ketoisovalerate Dehydrogenase. alpha-Ketoisovalerate Dehydrogenase. Tree number(s): ... Acylating) - Preferred Concept UI. M0077427. Scope note. An NAD+ dependent enzyme that catalyzes the oxidation 3-methyl-2- ... Dehydrogenase, 2-Oxoisovalerate Dehydrogenase, alpha-Ketoisovalerate alpha Ketoisovalerate Dehydrogenase alpha-Ketoisovalerate ...
EC 1.2.1.16 succinate-semialdehyde dehydrogenase [NAD(P)+]. EC 1.2.1.17 glyoxylate dehydrogenase (acylating). EC 1.2.1.18 ... EC 1.2.1.42 hexadecanal dehydrogenase (acylating). EC 1.2.1.43 formate dehydrogenase (NADP+). EC 1.2.1.44 cinnamoyl-CoA ... NAD-linked formate dehydrogenase; NAD-dependent formate dehydrogenase; formate dehydrogenase (NAD); NAD-formate dehydrogenase; ... Other name(s): MSDH; MMSA dehydrogenase; iolA (gene name); methylmalonate-semialdehyde dehydrogenase (acylating). Systematic ...
Dehydrogenase, alpha-Ketoglutarate .. alpha Ketoglutarate Dehydrogenase .. alpha Ketoglutarate Dehydrogenase Complex .. 0.77. 0 ... Acylating) .. 2-Oxoisovalerate Dehydrogenase .. alpha-Ketoisovalerate Dehydrogenase .. 2 Oxoisovalerate Dehydrogenase .. ... Dehydrogenase, Alpha-Keto Acid .. Dehydrogenase, Branched-Chain Oxo-Acid .. Oxo-Acid Dehydrogenase, Branched-Chain .. 2- ... Oxoisovalerate Dehydrogenase (Lipoamide) .. A ketone oxidoreductase that catalyzes the overall conversion of alpha-keto acids ...
Butanal dehydrogenase 1.2.1.58 Phenylglyoxylate dehydrogenase (acylating) 1.2.1.59 Glyceraldehyde-3-phosphate dehydrogenase ( ... CoA acylating) 1.2.1.28 Benzaldehyde dehydrogenase (NAD(+)) 1.2.1.29 Aryl-aldehyde dehydrogenase 1.2.1.30 Aryl-aldehyde ... Glutamate-5-semialdehyde dehydrogenase 1.2.1.42 Hexadecanal dehydrogenase (acylating) 1.2.1.43 Transferred entry: 1.17.1.10 1.2 ... Succinate-semialdehyde dehydrogenase (NAD(P)(+)) 1.2.1.17 Glyoxylate dehydrogenase (acylating) 1.2.1.18 Malonate-semialdehyde ...
... methylmalonate-semialdehyde dehydrogenase (acylating) MeSH D08.811.682.657.163.835 - retinal dehydrogenase MeSH D08.811.682.657 ... malate dehydrogenase MeSH D08.811.682.047.748 - malate dehydrogenase (nadp+) MeSH D08.811.682.047.892 - xanthine dehydrogenase ... acetoin dehydrogenase MeSH D08.811.682.047.070 - alcohol dehydrogenase MeSH D08.811.682.047.150 - carbohydrate dehydrogenases ... acylating) MeSH D08.811.682.657.350.875 - pyruvate dehydrogenase (lipoamide) MeSH D08.811.682.657.350.937 - pyruvate oxidase ...
3 beta-Hydroxy-delta-5-Steroid Dehydrogenase use Progesterone Reductase 3-beta-Hydroxysteroid Dehydrogenase use 3- ... Acylating) 2-Oxoisovalerate Dehydrogenase (Lipoamide) use 3-Methyl-2-Oxobutanoate Dehydrogenase (Lipoamide) ... 5,10-Methylenetetrahydrofolate-Reductase (NADH) use Methylenetetrahydrofolate Dehydrogenase (NAD+) 5,12-DiHETE use Leukotriene ... 3-Keto-5-alpha-Steroid delta-4-Dehydrogenase use Testosterone 5-alpha-Reductase ...
3-Keto-5-alpha-Steroid delta-4-Dehydrogenase use 3-Oxo-5-alpha-Steroid 4-Dehydrogenase ... Acylating) 2-Oxoisovalerate Dehydrogenase (Lipoamide) use 3-Methyl-2-Oxobutanoate Dehydrogenase (Lipoamide) ... 5,10-Methylenetetrahydrofolate-Reductase (NADH) use Methylenetetrahydrofolate Dehydrogenase (NAD+) 5,12-diHETE use Leukotriene ... 5-alpha-Reductase, Testosterone use 3-Oxo-5-alpha-Steroid 4-Dehydrogenase ...
Dehydrogenase (Acylating). 2-Oxoisovalerate Dehydrogenase (Lipoamide) use 3-Methyl-2-Oxobutanoate Dehydrogenase (Lipoamide) ... 3-Keto-5-alpha-Steroid delta-4-Dehydrogenase use Testosterone 5-alpha-Reductase ... 2H-Benzo(a)quinolizin-2-ol, 2-Ethyl-1,3,4,6,7,11b-hexahydro-3-isobutyl-9,10-dimethoxy- ... 3-Pyridinecarboxylic acid, 1,4-dihydro-2,6-dimethyl-5-nitro-4-(2-(trifluoromethyl)phenyl)-, Methyl ester ...
Acylating) *2-oxopyrrolidine *2-PAM *2-pentendoic acid Find out more. Search to find out more about Hereditary sensory and ... 2-Naphthylamine *2-oxo-5-guanidovaleric acid *2-oxoadipic acid *2-Oxohexane overdose *2-oxohexanedioic acid *2-Oxoisovalerate ... Dehydrogenase ( ...
DISPLAYABLE TEXT: 3-hydroxyacyl-coenzyme A dehydrogenase DISPLAYABLE TEXT: 3-hydroxyacyl-coenzyme A dehydrogenase (substance) ... acylating) DISPLAYABLE TEXT: 2-pivaloylindan-1,3-dione DISPLAYABLE TEXT: 2-pyridinol DISPLAYABLE TEXT: 2-pyridone DISPLAYABLE ... DISPLAYABLE TEXT: 3-hydroxybutyrate dehydrogenase DISPLAYABLE TEXT: 3-hydroxybutyrate dehydrogenase measurement DISPLAYABLE ... DISPLAYABLE TEXT: 3-hydroxybutyryl-coenzyme A dehydrogenase DISPLAYABLE TEXT: 3-hydroxybutyryl-coenzyme A dehydrogenase ( ...
Methylmalonate-semialdehyde dehydrogenase [acylating], mitochondrial. Q02252 ALDH6A1. 14q24.3. Unknown. Not Available. ... Isovaleryl-CoA dehydrogenase, mitochondrial. P26440 IVD. 15q14-q15. Unknown. Not Available. HMDBP00192. Peroxisomal acyl- ... Glutaryl-CoA dehydrogenase, mitochondrial. Q92947 GCDH. 19p13.2. Unknown. Not Available. HMDBP00226. Hepatic triacylglycerol ... Long-chain specific acyl-CoA dehydrogenase, mitochondrial. P28330 ACADL. 2q34. Unknown. Not Available. ...
oxoisovalerate. dehydrogenase. subunit alpha,. mitochondrial. Dihydrolipoyl. dehydrogenase,. mitochondrial. ... dehydrogenase. [acylating],. mitochondrial. Propionyl-CoA. carboxylase. alpha chain,. mitochondrial. Propionyl-CoA. carboxylase ... 2. O. O. 2. Methylmalonic. acid. Hydrogen. peroxide. NAD. H. 2. O. NADH. H. 2. O. Acetoacetyl-CoA. H. 2. O. CoA. CoA. Succinyl- ... 2. O. ATP. Hydrogen. carbonate. 3-Methylglutaconyl-CoA. ADP. Dihydrolipoamide. NAD. NADH. H. +. CoA. Isobutyryl-CoA. CO. 2. CO ...
  • In enzymology, a 2-oxoisovalerate dehydrogenase (acylating) (EC 1.2.1.25) is an enzyme that catalyzes the chemical reaction 3-methyl-2-oxobutanoate + CoA + NAD+ ⇌ {\displaystyle \rightleftharpoons } 2-methylpropanoyl-CoA + CO2 + NADH The 3 substrates of this enzyme are 3-methyl-2-oxobutanoate, CoA, and NAD+, whereas its 3 products are 2-methylpropanoyl-CoA, CO2, and NADH. (wikipedia.org)
  • carbon-monoxide dehydrogenase (cytochrome b -561). (qmul.ac.uk)
  • 2. Knox, W.E. The quinine-oxidizing enzyme and liver aldehyde oxidase. (qmul.ac.uk)
  • 2. Quayle, J.R. Formate dehydrogenase. (qmul.ac.uk)
  • 3. Jollie, D.R. and Lipscomb, J.D. Formate dehydrogenase from Methylosinus trichosporium OB3b. (qmul.ac.uk)
  • The systematic name of this enzyme class is 3-methyl-2-oxobutanoate:NAD+ 2-oxidoreductase (CoA-methyl-propanoylating). (wikipedia.org)
  • 1. Davison, D.C. Studies on plant formic dehydrogenase. (qmul.ac.uk)
  • An NAD+ dependent enzyme that catalyzes the oxidation 3-methyl-2-oxobutanoate to 2-methylpropanoyl-CoA. (bvsalud.org)
  • Coenzyme A- and nicotinamide adenine dinucleotide-dependent branched chain alpha-keto acid dehydrogenase. (wikipedia.org)
  • Other names in common use include 2-oxoisovalerate dehydrogenase, and alpha-ketoisovalerate dehydrogenase. (wikipedia.org)
  • Together with EC 1.12.1.2 hydrogen dehydrogenase, forms a system previously known as formate hydrogenlyase. (qmul.ac.uk)