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NameHydroxymethylglutaryl-CoA synthase, mitochondrial
Synonyms
  • 2.3.3.10
  • 3-hydroxy-3-methylglutaryl coenzyme A synthase
  • HMG-CoA synthase
Gene NameHMGCS2
OrganismHuman
Amino acid sequence
>lcl|BSEQ0009229|Hydroxymethylglutaryl-CoA synthase, mitochondrial
MQRLLTPVKRILQLTRAVQETSLTPARLLPVAHQRFSTASAVPLAKTDTWPKDVGILALE
VYFPAQYVDQTDLEKYNNVEAGKYTVGLGQTRMGFCSVQEDINSLCLTVVQRLMERIQLP
WDSVGRLEVGTETIIDKSKAVKTVLMELFQDSGNTDIEGIDTTNACYGGTASLFNAANWM
ESSSWDGRYAMVVCGDIAVYPSGNARPTGGAGAVAMLIGPKAPLALERGLRGTHMENVYD
FYKPNLASEYPIVDGKLSIQCYLRALDRCYTSYRKKIQNQWKQAGSDRPFTLDDLQYMIF
HTPFCKMVQKSLARLMFNDFLSASSDTQTSLYKGLEAFGGLKLEDTYTNKDLDKALLKAS
QDMFDKKTKASLYLSTHNGNMYTSSLYGCLASLLSHHSAQELAGSRIGAFSYGSGLAASF
FSFRVSQDAAPGSPLDKLVSSTSDLPKRLASRKCVSPEEFTEIMNQREQFYHKVNFSPPG
DTNSLFPGTWYLERVDEQHRRKYARRPV
Number of residues508
Molecular Weight56634.915
Theoretical pINot Available
GO Classification
Functions
  • hydroxymethylglutaryl-CoA synthase activity
Processes
  • response to nutrient
  • adipose tissue development
  • cholesterol biosynthetic process
  • response to ethanol
  • response to temperature stimulus
  • lung development
  • midgut development
  • response to growth hormone
  • kidney development
  • response to starvation
  • response to prostaglandin F
  • cellular response to insulin stimulus
  • cellular response to glucocorticoid stimulus
  • response to linoleic acid
  • cellular lipid metabolic process
  • response to glucagon
  • response to drug
  • response to monosaccharide
  • cellular response to lipopolysaccharide
  • isoprenoid biosynthetic process
  • response to triglyceride
  • brain development
  • multicellular organismal response to stress
  • cellular ketone body metabolic process
  • response to testosterone
  • small molecule metabolic process
  • ketone body biosynthetic process
  • response to metal ion
  • cellular response to amino acid stimulus
  • liver development
  • cellular response to fatty acid
  • response to cAMP
Components
  • mitochondrion
  • mitochondrial inner membrane
  • mitochondrial matrix
General FunctionHydroxymethylglutaryl-coa synthase activity
Specific FunctionThis enzyme condenses acetyl-CoA with acetoacetyl-CoA to form HMG-CoA, which is the substrate for HMG-CoA reductase.
Pfam Domain Function
Transmembrane RegionsNot Available
GenBank Protein IDNot Available
UniProtKB IDP54868
UniProtKB Entry NameHMCS2_HUMAN
Cellular LocationMitochondrion
Gene sequence
>lcl|BSEQ0021728|Hydroxymethylglutaryl-CoA synthase, mitochondrial (HMGCS2)
ATGCAGCGTCTGTTGACTCCAGTGAAGCGCATTCTGCAACTGACAAGAGCGGTGCAGGAA
ACCTCCCTCACACCTGCTCGCCTGCTCCCAGTAGCCCACCAAAGGTTTTCTACAGCCTCT
GCTGTCCCCCTGGCCAAAACAGATACTTGGCCAAAGGACGTGGGCATCCTGGCCCTGGAG
GTCTACTTCCCAGCCCAATATGTGGACCAAACTGACCTGGAGAAGTATAACAATGTGGAA
GCAGGAAAGTATACAGTGGGCTTGGGCCAGACCCGTATGGGCTTCTGCTCAGTCCAAGAG
GACATCAACTCCCTGTGCCTGACGGTGGTGCAACGGCTGATGGAGCGCATACAGCTCCCA
TGGGACTCTGTGGGCAGGCTGGAAGTAGGCACTGAGACCATCATTGACAAGTCCAAAGCT
GTCAAAACAGTGCTCATGGAACTCTTCCAGGATTCAGGCAATACTGATATTGAGGGCATA
GATACCACCAATGCCTGCTACGGTGGTACTGCCTCCCTCTTCAATGCTGCCAACTGGATG
GAGTCCAGTTCCTGGGATGGGCTGAGGGGAACCCATATGGAGAATGTGTATGACTTCTAC
AAACCAAATTTGGCCTCGGAGTACCCAATAGTGGATGGGAAGCTTTCCATCCAGTGCTAC
TTGCGGGCCTTGGATCGATGTTACACATCATACCGTAAAAAAATCCAGAATCAGTGGAAG
CAAGCTGGCAGCGATCGACCCTTCACCCTTGACGATTTACAGTACATGATCTTTCATACA
CCCTTTTGCAAGATGGTCCAGAAGTCTCTGGCTCGCCTGATGTTCAATGACTTCCTGTCA
GCCAGCAGTGACACACAAACCAGCTTATATAAGGGGCTGGAGGCTTTCGGGGGGCTAAAG
CTGGAAGACACCTACACCAACAAGGACCTGGATAAAGCACTTCTAAAGGCCTCTCAGGAC
ATGTTCGACAAGAAAACCAAGGCTTCCCTTTACCTCTCCACTCACAATGGGAACATGTAC
ACCTCATCCCTGTACGGGTGCCTGGCCTCGCTTCTGTCCCACCACTCTGCCCAAGAACTG
GCTGGCTCCAGGATTGGTGCCTTCTCTTATGGCTCTGGTTTAGCAGCAAGTTTCTTTTCA
TTTCGAGTATCCCAGGATGCTGCTCCAGGCTCTCCCCTGGACAAGTTGGTGTCCAGCACA
TCAGACCTGCCAAAACGCCTAGCCTCCCGAAAGTGTGTGTCTCCTGAGGAGTTCACAGAA
ATAATGAACCAAAGAGAGCAATTCTACCATAAGGTGAATTTCTCCCCACCTGGTGACACA
AACAGCCTTTTCCCAGGTACTTGGTACCTGGAGCGAGTGGACGAGCAGCATCGCCGAAAG
TATGCCCGGCGTCCCGTCTAA
GenBank Gene IDNot Available
GeneCard IDNot Available
GenAtlas IDNot Available
HGNC IDHGNC:5008
Chromosome Location1
LocusNot Available
References
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