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HMGCLL1

Function

Non-mitochondrial 3-hydroxymethyl-3-methylglutaryl-CoA lyase that catalyzes a cation-dependent cleavage of (S)-3-hydroxy-3-methylglutaryl-CoA into acetyl-CoA and acetoacetate, a key step in ketogenesis, the products of which support energy production in nonhepatic animal tissues.

Pathway

Metabolic intermediate metabolism; (S)-3-hydroxy-3-methylglutaryl-CoA degradation; acetoacetate from (S)-3-hydroxy-3-methylglutaryl-CoA: step 1/1.

Sequence Similarities

Belongs to the HMG-CoA lyase family.

Cellular localization

Alternative names

3-hydroxy-3-methylglutaryl-CoA lyase-like protein 1, Endoplasmic reticulum 3-hydroxy-3-methylglutaryl-CoA lyase, HMGCL-like 1, er-cHL, HMGCLL1

swissprot:Q8TB92 entrezGene:54511