HMGCS2

Available structures

Ortholog search: PDBe RCSB

List of PDB id codes
2WYA

Identifiers

Aliases

HMGCS2

External IDs

MGI: 101939 HomoloGene: 38066 GeneCards: HMGCS2

Gene ontology
Molecular function	• transferase activity • catalytic activity • hydroxymethylglutaryl-CoA synthase activity
Cellular component	• mitochondrial matrix • mitochondrion • mitochondrial inner membrane
Biological process	• multicellular organismal response to stress • steroid metabolic process • response to fatty acid • response to glucagon • response to monosaccharide • response to linoleic acid • sterol biosynthetic process • response to bacterium • response to organic cyclic compound • response to nutrient • kidney development • lung development • lipid metabolic process • response to testosterone • cellular response to organic cyclic compound • response to metal ion • response to peptide hormone • response to glucocorticoid • cholesterol metabolic process • isoprenoid biosynthetic process • response to insulin • ketone body biosynthetic process • cellular response to hormone stimulus • brain development • response to prostaglandin F • cellular response to glucocorticoid stimulus • cellular response to fatty acid • response to growth hormone • cellular response to insulin stimulus • response to starvation • cellular response to amino acid stimulus • liver development • response to triglyceride • adipose tissue development • cholesterol biosynthetic process • response to cAMP • metabolic process • response to ethanol • cellular response to lipopolysaccharide • response to drug • response to temperature stimulus • steroid biosynthetic process • midgut development
Sources:Amigo / QuickGO

Orthologs

Species

Human

Mouse

Entrez


3158


15360

Ensembl


ENSG00000134240


ENSMUSG00000027875

UniProt


P54868


P54869

RefSeq (mRNA)


NM_001166107 NM_005518


NM_008256

RefSeq (protein)


NP_001159579.1 NP_005509.1


NP_032282.2

Location (UCSC)

Chr 1: 119.75 – 119.77 Mb

Chr 3: 98.28 – 98.31 Mb

PubMed search

^[1]

^[2]

Wikidata

View/Edit Human

View/Edit Mouse

3-hydroxy-3-methylglutaryl-CoA synthase 2 (mitochondrial) is an enzyme in humans that is encoded by the HMGCS2 gene.^[3]

The protein encoded by this gene belongs to the HMG-CoA synthase family. It is a mitochondrial enzyme that catalyzes the first reaction of ketogenesis, a metabolic pathway that provides lipid-derived energy for various organs during times of carbohydrate deprivation, such as fasting. Mutations in this gene are associated with HMG-CoA synthase deficiency. Alternatively spliced transcript variants encoding different isoforms have been found for this gene.^[3]

References

Transferases: acyltransferases (EC 2.3)

2.3.1: other than amino-acyl groups	acetyltransferases: Acetyl-Coenzyme A acetyltransferase N-Acetylglutamate synthase Choline acetyltransferase Dihydrolipoyl transacetylase Acetyl-CoA C-acyltransferase Beta-galactoside transacetylase Chloramphenicol acetyltransferase N-acetyltransferase Serotonin N-acetyl transferase HGSNAT ARD1A Histone acetyltransferase P300/CBP NAT2 palmitoyltransferases: Carnitine O-palmitoyltransferase CPT1 CPT2 Serine C-palmitoyltransferase SPTLC1 SPTLC2 other: Acyltransferase like 2 Aminolevulinic acid synthase Beta-ketoacyl-ACP synthase Glyceronephosphate O-acyltransferase Lecithin—cholesterol acyltransferase Glycerol-3-phosphate O-acyltransferase 1-acylglycerol-3-phosphate O-acyltransferase 2-acylglycerol-3-phosphate O-acyltransferase ABHD5

2.3.2: Aminoacyltransferases	Gamma-glutamyl transpeptidase Peptidyl transferase Transglutaminase Tissue transglutaminase Keratinocyte transglutaminase Factor XIII

2.3.3: converted into alkyl on transfer	Citrate synthase ATP citrate lyase HMG-CoA synthase Malate synthase

Enzymes

Activity	Active site Binding site Catalytic triad Oxyanion hole Enzyme promiscuity Catalytically perfect enzyme Coenzyme Cofactor Enzyme catalysis Enzyme kinetics Lineweaver–Burk plot Michaelis–Menten kinetics

Regulation	Allosteric regulation Cooperativity Enzyme inhibitor

Classification	EC number Enzyme superfamily Enzyme family List of enzymes

Types	EC1 Oxidoreductases (list) EC2 Transferases (list) EC3 Hydrolases (list) EC4 Lyases (list) EC5 Isomerases (list) EC6 Ligases (list)

This article is issued from Wikipedia - version of the 5/20/2016. The text is available under the Creative Commons Attribution/Share Alike but additional terms may apply for the media files.