Identification
HMDB Protein ID CDBP00038
Secondary Accession Numbers Not Available
Name Hydroxymethylglutaryl-CoA synthase, mitochondrial
Description Not Available
Synonyms
  1. 3-hydroxy-3-methylglutaryl coenzyme A synthase
  2. HMG-CoA synthase
Gene Name HMGCS2
Protein Type Enzyme
Biological Properties
General Function Involved in hydroxymethylglutaryl-CoA synthase activity
Specific Function This enzyme condenses acetyl-CoA with acetoacetyl-CoA to form HMG-CoA, which is the substrate for HMG-CoA reductase.
GO Classification
Biological Process
cholesterol biosynthetic process
cellular response to fatty acid
cellular response to amino acid stimulus
lung development
ketone body biosynthetic process
cellular response to glucocorticoid stimulus
adipose tissue development
midgut development
response to testosterone stimulus
multicellular organismal response to stress
response to growth hormone stimulus
response to linoleic acid
response to monosaccharide stimulus
liver development
response to prostaglandin F stimulus
response to drug
response to temperature stimulus
response to ethanol
response to triglyceride
response to metal ion
cellular lipid metabolic process
response to nutrient
response to cAMP
response to starvation
cellular response to lipopolysaccharide
cellular response to insulin stimulus
isoprenoid biosynthetic process
brain development
response to glucagon stimulus
kidney development
Cellular Component
mitochondrial matrix
mitochondrial inner membrane
Function
transferase activity, transferring acyl groups
transferase activity, transferring acyl groups, acyl groups converted into alkyl on transfer
hydroxymethylglutaryl-coa synthase activity
catalytic activity
transferase activity
Molecular Function
hydroxymethylglutaryl-CoA synthase activity
Process
metabolic process
primary metabolic process
lipid metabolic process
cellular lipid metabolic process
isoprenoid metabolic process
isoprenoid biosynthetic process
Cellular Location
  1. Mitochondrion
Pathways
Gene Properties
Chromosome Location 1
Locus 1p13-p12
SNPs HMGCS2
Gene Sequence
>1527 bp
ATGCAGCGTCTGTTGACTCCAGTGAAGCGCATTCTGCAACTGACAAGAGCGGTGCAGGAA
ACCTCCCTCACACCTGCTCGCCTGCTCCCAGTAGCCCACCAAAGGTTTTCTACAGCCTCT
GCTGTCCCCCTGGCCAAAACAGATACTTGGCCAAAGGACGTGGGCATCCTGGCCCTGGAG
GTCTACTTCCCAGCCCAATATGTGGACCAAACTGACCTGGAGAAGTATAACAATGTGGAA
GCAGGAAAGTATACAGTGGGCTTGGGCCAGACCCGTATGGGCTTCTGCTCAGTCCAAGAG
GACATCAACTCCCTGTGCCTGACGGTGGTGCAACGGCTGATGGAGCGCATACAGCTCCCA
TGGGACTCTGTGGGCAGGCTGGAAGTAGGCACTGAGACCATCATTGACAAGTCCAAAGCT
GTCAAAACAGTGCTCATGGAACTCTTCCAGGATTCAGGCAATACTGATATTGAGGGCATA
GATACCACCAATGCCTGCTACGGTGGTACTGCCTCCCTCTTCAATGCTGCCAACTGGATG
GAGTCCAGTTCCTGGGATGGTCGTTATGCCATGGTGGTCTGTGGAGACATTGCCGTCTAT
CCCAGTGGTAATGCTCGTCCCACAGGTGGGGCCGGAGCTGTGGCTATGCTGATTGGGCCC
AAGGCCCCTCTGGCCCTGGAGCGAGGGCTGAGGGGAACCCATATGGAGAATGTGTATGAC
TTCTACAAACCAAATTTGGCCTCGGAGTACCCAATAGTGGATGGGAAGCTTTCCATCCAG
TGCTACTTGCGGGCCTTGGATCGATGTTACACATCATACCGTAAAAAAATCCAGAATCAG
TGGAAGCAAGCTGGCAGCGATCGACCCTTCACCCTTGACGATTTACAGTACATGATCTTT
CATACACCCTTTTGCAAGATGGTCCAGAAGTCTCTGGCTCGCCTGATGTTCAATGACTTC
CTGTCAGCCAGCAGTGACACACAAACCAGCTTATATAAGGGGCTGGAGGCTTTCGGGGGG
CTAAAGCTGGAAGACACCTACACCAACAAGGACCTGGATAAAGCACTTCTAAAGGCCTCT
CAGGACATGTTCGACAAGAAAACCAAGGCTTCCCTTTACCTCTCCACTCACAATGGGAAC
ATGTACACCTCATCCCTGTACGGGTGCCTGGCCTCGCTTCTGTCCCACCACTCTGCCCAA
GAACTGGCTGGCTCCAGGATTGGTGCCTTCTCTTATGGCTCTGGTTTAGCAGCAAGTTTC
TTTTCATTTCGAGTATCCCAGGATGCTGCTCCAGGCTCTCCCCTGGACAAGTTGGTGTCC
AGCACATCAGACCTGCCAAAACGCCTAGCCTCCCGAAAGTGTGTGTCTCCTGAGGAGTTC
ACAGAAATAATGAACCAAAGAGAGCAATTCTACCATAAGGTGAATTTCTCCCCACCTGGT
GACACAAACAGCCTTTTCCCAGGTACTTGGTACCTGGAGCGAGTGGACGAGCAGCATCGC
CGAAAGTATGCCCGGCGTCCCGTCTAA
Protein Properties
Number of Residues 508
Molecular Weight 52481.065
Theoretical pI 7.877
Pfam Domain Function
Signals Not Available
Transmembrane Regions Not Available
Protein Sequence
>Hydroxymethylglutaryl-CoA synthase, mitochondrial
MQRLLTPVKRILQLTRAVQETSLTPARLLPVAHQRFSTASAVPLAKTDTWPKDVGILALE
VYFPAQYVDQTDLEKYNNVEAGKYTVGLGQTRMGFCSVQEDINSLCLTVVQRLMERIQLP
WDSVGRLEVGTETIIDKSKAVKTVLMELFQDSGNTDIEGIDTTNACYGGTASLFNAANWM
ESSSWDGRYAMVVCGDIAVYPSGNARPTGGAGAVAMLIGPKAPLALERGLRGTHMENVYD
FYKPNLASEYPIVDGKLSIQCYLRALDRCYTSYRKKIQNQWKQAGSDRPFTLDDLQYMIF
HTPFCKMVQKSLARLMFNDFLSASSDTQTSLYKGLEAFGGLKLEDTYTNKDLDKALLKAS
QDMFDKKTKASLYLSTHNGNMYTSSLYGCLASLLSHHSAQELAGSRIGAFSYGSGLAASF
FSFRVSQDAAPGSPLDKLVSSTSDLPKRLASRKCVSPEEFTEIMNQREQFYHKVNFSPPG
DTNSLFPGTWYLERVDEQHRRKYARRPV
GenBank ID Protein 56205097
UniProtKB/Swiss-Prot ID P54868
UniProtKB/Swiss-Prot Entry Name HMCS2_HUMAN
PDB IDs
GenBank Gene ID AL589734
GeneCard ID HMGCS2
GenAtlas ID HMGCS2
HGNC ID HGNC:5008
References
General References Not Available