Names & Taxonomy
- Uniprot ID:
- P35914
- Entry Name:
- HMGCL_HUMAN
- Status:
- reviewed
- Protein Names:
- Hydroxymethylglutaryl-CoA lyase, mitochondrial (HL) (HMG-CoA lyase) (EC 4.1.3.4) (3-hydroxy-3-methylglutarate-CoA lyase)
- Gene Names:
- HMGCL
- Gene Names Primary:
- HMGCL
- Organism:
- Homo sapiens (Human)
Structure
- Length:
- 325
- Sequence:
- MAAMRKALPRRLVGLASLRAVSTSSMGTLPKRVKIVEVGPRDGLQNEKNIVSTPVKIKLIDMLSEAGLSVIETTSFVSPKWVPQMGDHTEVLKGIQKFPGINYPVLTPNLKGFEAAVAAGAKEVVIFGAASELFTKKNINCSIEESFQRFDAILKAAQSANISVRGYVSCALGCPYEGKISPAKVAEVTKKFYSMGCYEISLGDTIGVGTPGIMKDMLSAVMQEVPLAALAVHCHDTYGQALANTLMALQMGVSVVDSSVAGLGGCPYAQGASGNLATEDLVYMLEGLGIHTGVNLQKLLEAGNFICQALNRKTSSKVAQATCKL
- Proteomes:
- UP000005640
Subcellular location
- Subcellular Location:
- Mitochondrion matrix. Peroxisome. Note=Unprocessed form is peroxisomal.
Function
- Function:
- Key enzyme in ketogenesis (ketone body formation). Terminal step in leucine catabolism. Ketone bodies (beta-hydroxybutyrate, acetoacetate and acetone) are essential as an alternative source of energy to glucose, as lipid precursors and as regulators of metabolism.
- Pathway:
- Metabolic intermediate metabolism; (S)-3-hydroxy-3-methylglutaryl-CoA degradation; acetoacetate from (S)-3-hydroxy-3-methylglutaryl-CoA: step 1/1.
- Catalytic Activity:
- (S)-3-hydroxy-3-methylglutaryl-CoA = acetyl-CoA + acetoacetate.
- Cofactor:
- COFACTOR: Name=a divalent metal cation; Xref=ChEBI:CHEBI:60240;
- Kinetics:
- BIOPHYSICOCHEMICAL PROPERTIES: Kinetic parameters: KM=200 uM for magnesium ion
- Enzyme Regulation:
- ENZYME REGULATION: Stimulated by reducing agents such as dithiothreitol (DTT).
- Active Site:
- ACT_SITE 266 266
- Gene Ontology Go:
- mitochondrial inner membrane
mitochondrial matrix
mitochondrion
peroxisome
carboxylic acid binding
fatty-acyl-CoA binding
hydroxymethylglutaryl-CoA lyase activity
magnesium ion binding
manganese ion binding
metal ion binding
protein homodimerization activity
receptor binding
acyl-CoA metabolic process
cellular ketone body metabolic process
cellular lipid metabolic process
ketone body biosynthetic process
leucine catabolic process
liver development
mitochondrion organization
protein tetramerization
response to fatty acid
response to nutrient
response to starvation
small molecule metabolic process - Gene Ontology Biological Process:
- acyl-CoA metabolic process
cellular ketone body metabolic process
cellular lipid metabolic process
ketone body biosynthetic process
leucine catabolic process
liver development
mitochondrion organization
protein tetramerization
response to fatty acid
response to nutrient
response to starvation
small molecule metabolic process - Gene Ontology Molecular Function:
- carboxylic acid binding
fatty-acyl-CoA binding
hydroxymethylglutaryl-CoA lyase activity
magnesium ion binding
manganese ion binding
metal ion binding
protein homodimerization activity
receptor binding - Gene Ontology Cellular Component:
- mitochondrial inner membrane
mitochondrial matrix
mitochondrion
peroxisome - Keywords:
- 3D-structure
Acetylation
Alternative splicing
Complete proteome
Disease mutation
Disulfide bond
Lyase
Metal-binding
Mitochondrion
Peroxisome
Reference proteome
Transit peptide