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3-hydroxy-3-methylglutaryl-CoA lyase: Wikis

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3-hydroxymethyl-3-methylglutaryl-Coenzyme A lyase (hydroxymethylglutaricaciduria)
Identifiers
Symbol HMGCL
Entrez 3155
HUGO 5005
OMIM 246450
RefSeq NM_000191
UniProt P35914
Other data
EC number 4.1.3.4
Locus Chr. 1 p36.1-p35

3-hydroxy-3-methylglutaryl-CoA lyase (or HMG-CoA lyase) plays an essential role in breaking down dietary proteins and fats for energy.

Specifically, the enzyme is responsible for processing the amino acid leucine.

Pathology

Mutations in the HMGCL gene cause 3-hydroxy-3-methylglutaryl-CoA lyase deficiency.

External links

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