HMGCS2

3-hydroxy-3-methylglutaryl-CoA synthase 2

Available structures

Ortholog search: PDBe, RCSB

List of PDB id codes
2WYA

Identifiers

Symbol

HMGCS2

External IDs

OMIM: 600234 HomoloGene: 38066 IUPHAR: 2432 GeneCards: HMGCS2 Gene

EC number

2.3.3.10

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

Orthologs

Species

Human

Mouse

RefSeq (mRNA)

RefSeq (protein)

Location (UCSC)

Chr 1:
119.75 – 119.77 Mb

Chr 3:
98.28 – 98.31 Mb

PubMed search

3-hydroxy-3-methylglutaryl-CoA synthase 2 (mitochondrial) is an enzyme in humans that is encoded by the HMGCS2 gene.^[1]

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.^[1]

References

1 2 "Entrez Gene: 3-hydroxy-3-methylglutaryl-CoA synthase 2 (mitochondrial)".

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

Proteins: 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)

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