1.1.1.178: 3-hydroxy-2-methylbutyryl-CoA dehydrogenase

This is an abbreviated version, for detailed information about 3-hydroxy-2-methylbutyryl-CoA dehydrogenase, go to the full flat file.

Reaction

(2S,3S)-3-hydroxy-2-methylbutanoyl-CoA
+
NAD+
=
2-methylacetoacetyl-CoA
+
NADH
+
H+

Synonyms

17beta-HSD10, 2-methyl-3-hydroxy-butyryl CoA dehydrogenase, 2-methyl-3-hydroxybutyryl coenzyme A dehydrogenase, 2-methyl-3-hydroxybutyryl-CoA dehydrogenase, 3-hydroxyacyl-CoA dehydrogenase type II, dehydrogenase, 2-methyl-3-hydroxybutyryl coenzyme A, HSD10, MHBD, short-chain L-3-hydroxy-2-methylacyl-CoA dehydrogenase

ECTree

     1 Oxidoreductases
         1.1 Acting on the CH-OH group of donors
             1.1.1 With NAD+ or NADP+ as acceptor
                1.1.1.178 3-hydroxy-2-methylbutyryl-CoA dehydrogenase

Reference

Reference on EC 1.1.1.178 - 3-hydroxy-2-methylbutyryl-CoA dehydrogenase

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REF.
AUTHORS
TITLE
JOURNAL
VOL.
PAGES
YEAR
ORGANISM (UNIPROT)
PUBMED ID
SOURCE
Conrad R.S.; Massey L.K.; Sokatch J.R.
D- and L-leucine metabolism and regulation of their pathways in Pseudomonas putida
J. Bacteriol.
4
103-111
1974
Pseudomonas putida, Pseudomonas putida PpG2
Manually annotated by BRENDA team
Gibson, K.M.; Lee, C.F.; Kamali, V.; Soevik, O.
A coupled assay detecting defects in fibroblasts isoleucine degradation distal to enoyl-CoA hydratase: application to 3-oxothiolase deficiency
Clin. Chim. Acta
205
127-135
1992
Homo sapiens
Manually annotated by BRENDA team
Luo, M.J.; Mao, L.F.; Schulz, H.
Short-chain 3-hydroxy-2-methylacyl-CoA dehydrogenase from rat liver: purification and characterization of a novel enzyme of isoleucine metabolism
Arch. Biochem. Biophys.
321
214-220
1995
Rattus norvegicus
Manually annotated by BRENDA team
Zschocke, J.; Ruiter, J.P.N.; Brand, J.; Lindner, M.; Hoffmann, G.F.; Wanders, R.J.A.; Mayatepek, E.
Progressive infantile neurodegeneration caused by 2-methyl-3-hydroxybutyryl-CoA dehydrogenase deficiency: a novel inborn error of branched-chain fatty acid and isoleucine metabolism
Pediatr. Res.
48
852-855
2000
Homo sapiens
Manually annotated by BRENDA team
Garcia-Villoria, J.; Ofman, R.; Sala, P.R.; Merinero, B.; Ramos, J.; Garcia-Silva, M.T.; Beseler, B.; Dalmau, J.; Wanders, R.J.; Ugarte, M.
2-Methyl-3-hydroxybutyryl-CoA dehydrogenase (MHBD) deficiency: an X-linked inborn error of isoleucine metabolism that may mimic a mitochondrial disease
Pediatr. Res.
58
488-491
2005
Homo sapiens
Manually annotated by BRENDA team
Garcia-Villoria, J.; Navarro-Sastre, A.; Fons, C.; Perez-Cerda, C.; Baldellou, A.; Fuentes-Castello, M.A.; Gonzalez, I.; Hernandez-Gonzalez, A.; Fernandez, C.; Campistol, J.; Delpiccolo, C.; Cortes, N.; Messeguer, A.; Briones, P.; Ribes, A.
Study of patients and carriers with 2-methyl-3-hydroxybutyryl-CoA dehydrogenase (MHBD) deficiency: difficulties in the diagnosis
Clin. Biochem.
42
27-33
2009
Homo sapiens
Manually annotated by BRENDA team
Rauschenberger, K.; Schoeler, K.; Sass, J.O.; Sauer, S.; Djuric, Z.; Rumig, C.; Wolf, N.I.; Okun, J.G.; Koelker, S.; Schwarz, H.; Fischer, C.; Grziwa, B.; Runz, H.; Nuemann, A.; Shafqat, N.; Kavanagh, K.L.; Haemmerling, G.; Wanders, R.J.; Shield, J.P.; Wendel, U.; Stern, D.; Nawroth, P.; Hoffmann, G.F.; Bartram, C.R.
A non-enzymatic function of 17beta-hydroxysteroid dehydrogenase type 10 is required for mitochondrial integrity and cell survival
EMBO Mol. Med.
2
51-62
2010
Homo sapiens, Homo sapiens (Q99714), Mus musculus, Xenopus laevis
Manually annotated by BRENDA team
Yang, S.Y.; Dobkin, C.; He, X.Y.; Philipp, M.; Brown, W.T.
A 5-methylcytosine hotspot responsible for the prevalent HSD17B10 mutation
Gene
515
380-384
2013
Homo sapiens, Homo sapiens (Q99714)
Manually annotated by BRENDA team