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

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

J Mol Biol 2004
12696021 2-Methyl-3-hydroxybutyryl-CoA dehydrogenase deficiency is caused by mutations in the HADH2 gene.

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

Am J Hum Genet 2003
Catalyst Activity

3-hydroxyacyl-CoA dehydrogenase activity of HSD17B10 tetramer [mitochondrial matrix]

Orthologous Events
Cross References
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