Hydroxymethylglutaryl-CoA lyase, mitochondrial

NameHydroxymethylglutaryl-CoA lyase, mitochondrial
Synonyms
  • 3-hydroxy-3-methylglutarate-CoA lyase
  • 4.1.3.4
  • HL
Gene NameHMGCL
OrganismHuman
Amino acid sequence
>lcl|BSEQ0006501|Hydroxymethylglutaryl-CoA lyase, mitochondrial
MAAMRKALPRRLVGLASLRAVSTSSMGTLPKRVKIVEVGPRDGLQNEKNIVSTPVKIKLI
DMLSEAGLSVIETTSFVSPKWVPQMGDHTEVLKGIQKFPGINYPVLTPNLKGFEAAVAAG
AKEVVIFGAASELFTKKNINCSIEESFQRFDAILKAAQSANISVRGYVSCALGCPYEGKI
SPAKVAEVTKKFYSMGCYEISLGDTIGVGTPGIMKDMLSAVMQEVPLAALAVHCHDTYGQ
ALANTLMALQMGVSVVDSSVAGLGGCPYAQGASGNLATEDLVYMLEGLGIHTGVNLQKLL
EAGNFICQALNRKTSSKVAQATCKL
Number of residues325
Molecular Weight34359.84
Theoretical pI8.71
GO Classification
Functions
  • protein homodimerization activity
  • receptor binding
  • fatty-acyl-CoA binding
  • metal ion binding
  • manganese ion binding
  • carboxylic acid binding
  • magnesium ion binding
  • hydroxymethylglutaryl-CoA lyase activity
Processes
  • protein tetramerization
  • leucine catabolic process
  • cellular lipid metabolic process
  • mitochondrion organization
  • cellular ketone body metabolic process
  • ketone body biosynthetic process
  • liver development
  • response to nutrient
  • response to starvation
  • response to fatty acid
  • acyl-CoA metabolic process
  • small molecule metabolic process
Components
  • mitochondrion
  • mitochondrial inner membrane
  • mitochondrial matrix
  • peroxisome
General FunctionReceptor binding
Specific FunctionKey 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.
Pfam Domain Function
Transmembrane RegionsNot Available
GenBank Protein IDNot Available
UniProtKB IDP35914
UniProtKB Entry NameHMGCL_HUMAN
Cellular LocationMitochondrion matrix
Gene sequence
>lcl|BSEQ0019402|Hydroxymethylglutaryl-CoA lyase, mitochondrial (HMGCL)
ATGGCAGCAATGAGGAAGGCGCTTCCGCGGCGACTGGTGGGCTTGGCGTCCCTCCGGGCT
GTCAGCACCTCATCTATGGGCACTTTACCAAAGCGGGTGAAAATTGTGGAAGTTGGTCCC
CGAGATGGACTACAAAATGAAAAGAATATCGTATCTACTCCAGTGAAAATCAAGCTGATA
GACATGCTTTCTGAAGCAGGACTCTCTGTTATAGAAACCACCAGCTTTGTGTCTCCTAAG
TGGGTTCCCCAGATGGGTGACCACACTGAAGTCTTGAAGGGCATTCAGAAGTTTCCTGGC
ATCAACTACCCAGTCCTGACCCCAAATTTGAAAGGCTTCGAGGCAGCGGTTGCTGCTGGA
GCCAAGGAAGTAGTCATCTTTGGAGCTGCCTCAGAGCTCTTCACCAAGAAGAACATCAAT
TGTTCCATAGAGGAGAGTTTTCAGAGGTTTGACGCAATCCTGAAGGCAGCGCAGTCAGCC
AATATTTCTGTGCGGGGGTACGTCTCCTGTGCTCTTGGCTGCCCTTATGAAGGGAAGATC
TCCCCAGCTAAAGTAGCTGAGGTCACCAAGAAGTTCTACTCAATGGGCTGCTACGAGATC
TCCCTGGGGGACACCATTGGTGTGGGCACCCCAGGGATCATGAAAGACATGCTATCTGCT
GTCATGCAGGAAGTGCCTCTGGCTGCCCTGGCTGTCCACTGCCATGACACCTATGGTCAA
GCCCTGGCCAACACCTTGATGGCCCTGCAGATGGGAGTGAGTGTCGTGGACTCTTCTGTG
GCAGGACTTGGAGGCTGTCCCTACGCACAGGGGGCATCAGGAAACTTGGCCACAGAAGAC
CTGGTCTACATGCTAGAGGGCTTGGGCATTCACACGGGTGTGAATCTCCAGAAGCTTCTG
GAAGCTGGAAACTTTATCTGTCAAGCCCTGAACAGAAAAACTAGCTCCAAAGTGGCTCAG
GCTACCTGTAAACTCTGA
GenBank Gene IDL07033
GeneCard IDNot Available
GenAtlas IDHMGCL
HGNC IDHGNC:5005
Chromosome Location1
Locus1p36.1-p35
References
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