alpha-methyl-beta-hydroxybutyryl-CoA + NAD+ alpha-methylacetoacetyl-CoA + NADH + H+

aMbHBUT-CoA + NAD+ → 2MACA-CoA + NADH + H+

Reactome

R-HSA-70837

Compartment

mitochondrial matrix

Rhea

RHEA:13282

Catalysed by

• (3S)-3-hydroxyacyl-CoA dehydrogenase (NAD+) activity of HSD17B10 tetramer [mitochondrial matrix]EC 1.1.1.35

Literature

1. Crystal structure of human ABAD/HSD10 with a bound inhibitor: implications for design of Alzheimer's disease therapeutics J Mol Biol, 2004
2. 2-Methyl-3-hydroxybutyryl-CoA dehydrogenase deficiency is caused by mutations in the HADH2 gene. Am J Hum Genet, 2003