alpha-methyl-beta-hydroxybutyryl-CoA + NAD+ <=> alpha-methylacetoacetyl-CoA + NADH + H+

Stable Identifier
Reaction [transition]
Homo sapiens
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Mitochondrial 3-hydroxyacyl-CoA dehydrogenase type-2 (HSD17B10; HADH2) catalyzes the reversible reaction of alpha-methyl-beta-hydroxybutyryl-CoA and NAD+ to form alpha-methylacetoacetyl-CoA and NADH + H+ (Ofman et al. 2003). Crystallographic data indicate that the enzyme is a homotetramer (Kissinger et al. 2004).

Literature References
PubMed ID Title Journal Year
12696021 2-Methyl-3-hydroxybutyryl-CoA dehydrogenase deficiency is caused by mutations in the HADH2 gene.

Ofman, R, Ruiter, JP, Feenstra, M, Duran, M, Poll-The, BT, Zschocke, J, Ensenauer, R, Lehnert, W, Sass, JO, Sperl, W

Am J Hum Genet 2003
15342248 Crystal structure of human ABAD/HSD10 with a bound inhibitor: implications for design of Alzheimer's disease therapeutics

Kissinger, CR, Rejto, PA, Pelletier, LA, Thomson, JA, Showalter, RE, Abreo, MA, Agree, CS, Margosiak, S, Meng, JJ, Aust, RM, Vanderpool, D, Li, B, Tempczyk-Russell, A, Villafranca, JE

J Mol Biol 2004
Participant Of
Event Information
Catalyst Activity
Catalyst Activity
3-hydroxyacyl-CoA dehydrogenase activity of HSD17B10 tetramer [mitochondrial matrix]
Physical Entity
Orthologous Events
Cross References
Target Pathogen
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