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

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

Stable Identifier

R-HSA-508369

Type

Reaction [transition]

Species

Homo sapiens

Compartment

mitochondrial matrix

Synonyms

2-methyl-3-oxobutanoyl-CoA + H(+) + NADH => (2S,3S)-3-hydroxy-2-methylbutanoyl-CoA + NAD(+)

ReviewStatus

5/5

Locations in the PathwayBrowser

Expand all

General

SBML | | PDF

SVG | | PPTX | SBGN

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

Click the image above or here to open this reaction in the Pathway Browser
The layout of this reaction may differ from that in the pathway view due to the constraints in pathway layout

Mitochondrial 3-hydroxyacyl-CoA dehydrogenase type-2 (HSD17B10; HADH2) catalyzes the reversible reaction of alpha-methylacetoacetyl-CoA and NADH + H+ to form alpha-methyl-beta-hydroxybutyryl-CoA and NAD+ (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