3-hydroxy-3-methylglutaryl-CoA lyase

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Hydroxymethylglutaryl-CoA lyase
Identifiers
EC number 4.1.3.4
CAS number 9030-83-5
Databases
IntEnz IntEnz view
BRENDA BRENDA entry
ExPASy NiceZyme view
KEGG KEGG entry
MetaCyc metabolic pathway
PRIAM profile
PDB structures RCSB PDB PDBe PDBsum
Gene Ontology AmiGO / EGO
3-hydroxymethyl-3-methylglutaryl-Coenzyme A lyase (hydroxymethylglutaricaciduria)
Identifiers
Symbol HMGCL
Entrez 3155
HUGO 5005
OMIM 246450
RefSeq NM_000191
UniProt P35914
Other data
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 required for ketogenesis in the liver, and is also responsible for processing the amino acid leucine. It is found in the mitochondrion.

Pathology[edit]

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

External links[edit]