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Identification
BMDB Protein ID BMDBP01139
Secondary Accession Numbers
  • BMDBP03046
Name Hydroxymethylglutaryl-CoA lyase, mitochondrial
Synonyms Not Available
Gene Name HMGCL
Protein Type Enzyme
Biological Properties
General Function Amino acid transport and metabolism
Specific Function Mitochondrial 3-hydroxymethyl-3-methylglutaryl-CoA lyase that catalyzes a cation-dependent cleavage of (S)-3-hydroxy-3-methylglutaryl-CoA into acetyl-CoA and acetoacetate, a key step in ketogenesis. 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.
Pathways
  • (S)-3-hydroxy-3-methylglutaryl-CoA degradation
  • Acetoacetate from (S)-3-hydroxy-3-methylglutaryl-CoA: step 1/1
  • Metabolic intermediate metabolism
Reactions
3-Hydroxy-3-methylglutaryl-CoA → Acetoacetic acid + Acetyl-CoA details
3-Hydroxy-3-methylglutaryl-CoA → Acetyl-CoA details
3-Hydroxy-3-methylglutaryl-CoA → Acetoacetic acid details
GO Classification Not Available
Cellular Location Not Available
Gene Properties
Chromosome Location 2
Locus Not Available
SNPs HMGCL
Gene Sequence
>978 bp
ATGGCGACAGTGAAGAAGGTGCTCCCGCGGAGGCTGGTGGGCTTGGCGACCCTCCGGGCT
GTCAGCACCTCATCTGTGGGTACGTTCCCAAAGCAAGTGAAAATTGTGGAAGTTGGTCCT
CGAGACGGACTACAAAATGAAAAGAATATTGTACCTACTCCAGTGAAAATCAAGCTAATA
GACATGCTTTCCGAAGCAGGACTCCCTGTGGTAGAAGCCACCAGCTTCGTGTCTCCCAAA
TGGGTTCCACAGATGGCTGACCATGCTGAAGTCTTGAAAGGCATCCAGAAATTTCCTGGC
GTCAACTACCCAGTCCTGACCCCAAACTTCAAAGGCTTCCAGGCAGCGGTTGCTGCCGGA
GCCAAGGAAGTGGCCATCTTTGGAGCCGCCTCTGAACTCTTCACCAAGAAGAACATCAAC
TGCTCCATAGATGAGAGTTTGCAGCGGTTTGATGAAATCTTGAAGGCAGCTCGGGCAGCC
GGTATCTCTGTGCGGGGGTACGTCTCCTGTGTGCTTGGATGTCCCTACGAAGGGAAGATC
TCCCCGGCTAAAGTCGCTGAGGTCACCAAGAAGCTGTACTCCATGGGCTGCTACGAGATC
TCCCTGGGGGACACCATCGGCGTGGGCACCCCTGGGGCCATGAAGGACATGCTGTCTGCT
GTCCTGCAGGAGGTGCCAGTGACAGCCCTGGCCGTCCACTGCCATGACACCTACGGCCAG
GCCCTGGCCAACACCTTGACGGCCCTGCAGATGGGAGTGAGTGTCATGGACTCTTCCGTG
GCAGGACTGGGAGGCTGTCCCTATGCACAGGGGGCGTCGGGAAACTTGGCCACCGAGGAC
CTGGTCTACATGCTGGCCGGCCTGGGCATTCACACGGGTGTGAACCTCCAGAAGCTTCTG
GAAGCTGGGGCCTTCATCTGTCAAGCCCTGAACAGAAGAACCAACTCCAAAGTGGCTCAG
GCTACCTGTAAACTGTGA
Protein Properties
Number of Residues 325
Molecular Weight 34168.0
Theoretical pI 8.88
Pfam Domain Function Not Available
Signals
  • None
Transmembrane Regions Not Available
Protein Sequence
>Hydroxymethylglutaryl-CoA lyase, mitochondrial
MATVKKVLPRRLVGLATLRAVSTSSVGTFPKQVKIVEVGPRDGLQNEKNIVPTPVKIKLI
DMLSEAGLPVVEATSFVSPKWVPQMADHAEVLKGIQKFPGVNYPVLTPNFKGFQAAVAAG
AKEVAIFGAASELFTKKNINCSIDESLQRFDEILKAARAAGISVRGYVSCVLGCPYEGKI
SPAKVAEVTKKLYSMGCYEISLGDTIGVGTPGAMKDMLSAVLQEVPVTALAVHCHDTYGQ
ALANTLTALQMGVSVMDSSVAGLGGCPYAQGASGNLATEDLVYMLAGLGIHTGVNLQKLL
EAGAFICQALNRRTNSKVAQATCKL
GenBank ID Protein AAI18277.1
UniProtKB/Swiss-Prot ID Q29448
UniProtKB/Swiss-Prot Entry Name HMGCL_BOVIN
PDB IDs Not Available
GenBank Gene ID BC118276
GeneCard ID HMGCL
GenAtlas ID Not Available
HGNC ID Not Available
References
General References Not Available