| Identification |
|---|
| BMDB Protein ID
| BMDBP01139 |
| Secondary Accession Numbers
| |
| 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
|
|
|
Transmembrane Regions
|
Not Available
|
| Protein Sequence
|
>Hydroxymethylglutaryl-CoA lyase, mitochondrial
MATVKKVLPRRLVGLATLRAVSTSSVGTFPKQVKIVEVGPRDGLQNEKNIVPTPVKIKLI
DMLSEAGLPVVEATSFVSPKWVPQMADHAEVLKGIQKFPGVNYPVLTPNFKGFQAAVAAG
AKEVAIFGAASELFTKKNINCSIDESLQRFDEILKAARAAGISVRGYVSCVLGCPYEGKI
SPAKVAEVTKKLYSMGCYEISLGDTIGVGTPGAMKDMLSAVLQEVPVTALAVHCHDTYGQ
ALANTLTALQMGVSVMDSSVAGLGGCPYAQGASGNLATEDLVYMLAGLGIHTGVNLQKLL
EAGAFICQALNRRTNSKVAQATCKL
|
| External Links |
|---|
| 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 |
