| Identification |
|---|
| HMDB Protein ID
| CDBP00009 |
| Secondary Accession Numbers
| Not Available |
| Name
| Hydroxymethylglutaryl-CoA lyase, mitochondrial |
| Description
| Not Available |
| Synonyms
|
- 3-hydroxy-3-methylglutarate-CoA lyase
- HL
- HMG-CoA lyase
|
| Gene Name
| HMGCL |
| Protein Type
| Enzyme |
| Biological Properties |
|---|
| General Function
| Involved in catalytic activity |
| Specific Function
| Key enzyme in ketogenesis (ketone body formation). Terminal step in leucine catabolism.
|
| GO Classification
|
| Biological Process |
| acyl-CoA metabolic process |
| embryo development |
| ketone body biosynthetic process |
| liver development |
| response to nutrient |
| response to starvation |
| protein tetramerization |
| response to fatty acid |
| mitochondrion organization |
| cellular lipid metabolic process |
| leucine catabolic process |
| Cellular Component |
| mitochondrial matrix |
| peroxisome |
| mitochondrial inner membrane |
| Function |
| catalytic activity |
| lyase activity |
| carbon-carbon lyase activity |
| oxo-acid-lyase activity |
| hydroxymethylglutaryl-coa lyase activity |
| Molecular Function |
| manganese ion binding |
| magnesium ion binding |
| carboxylic acid binding |
| protein homodimerization activity |
| hydroxymethylglutaryl-CoA lyase activity |
| fatty-acyl-CoA binding |
| Process |
| metabolic process |
|
| Cellular Location
|
- Mitochondrion matrix
|
| Pathways
|
| Name | SMPDB/Pathwhiz | KEGG | | (S)-3-hydroxy-3-methylglutaryl-CoA degradation | Not Available | Not Available | | Synthesis and degradation of ketone bodies | Not Available |  | | Butanoate metabolism | Not Available |  | | Peroxisome | Not Available |  | | Butyrate Metabolism |    | |
|
| Gene Properties |
|---|
| Chromosome Location
| 1 |
| Locus
| 1p36.1-p35 |
| SNPs
| HMGCL |
| Gene Sequence
|
>978 bp
ATGGCAGCAATGAGGAAGGCGCTTCCGCGGCGACTGGTGGGCTTGGCGTCCCTCCGGGCT
GTCAGCACCTCATCTATGGGCACTTTACCAAAGCGGGTGAAAATTGTGGAAGTTGGTCCC
CGAGATGGACTACAAAATGAAAAGAATATCGTATCTACTCCAGTGAAAATCAAGCTGATA
GACATGCTTTCTGAAGCAGGACTCTCTGTTATAGAAACCACCAGCTTTGTGTCTCCTAAG
TGGGTTCCCCAGATGGGTGACCACACTGAAGTCTTGAAGGGCATTCAGAAGTTTCCTGGC
ATCAACTACCCAGTCCTGACCCCAAATTTGAAAGGCTTCGAGGCAGCGGTTGCTGCTGGA
GCCAAGGAAGTAGTCATCTTTGGAGCTGCCTCAGAGCTCTTCACCAAGAAGAACATCAAT
TGTTCCATAGAGGAGAGTTTTCAGAGGTTTGACGCAATCCTGAAGGCAGCGCAGTCAGCC
AATATTTCTGTGCGGGGGTACGTCTCCTGTGCTCTTGGCTGCCCTTATGAAGGGAAGATC
TCCCCAGCTAAAGTAGCTGAGGTCACCAAGAAGTTCTACTCAATGGGCTGCTACGAGATC
TCCCTGGGGGACACCATTGGTGTGGGCACCCCAGGGATCATGAAAGACATGCTGTCTGCT
GTCATGCAGGAAGTGCCTCTGGCTGCCCTGGCTGTCCACTGCCATGACACCTATGGTCAA
GCCCTGGCCAACACCTTGATGGCCCTGCAGATGGGAGTGAGTGTCGTGGACTCTTCTGTG
GCAGGACTTGGAGGCTGTCCCTACGCACAGGGGGCATCAGGAAACTTGGCCACAGAAGAC
CTGGTCTACATGCTAGAGGGCTTGGGCATTCACACGGGTGTGAATCTCCAGAAGCTTCTG
GAAGCTGGAAACTTTATCTGTCAAGCCCTGAACAGAAAAACTAGCTCCAAAGTGGCTCAG
GCTACCTGTAAACTCTGA
|
| Protein Properties |
|---|
| Number of Residues
| 325 |
| Molecular Weight
| 34359.84 |
| Theoretical pI
| 8.545 |
| Pfam Domain Function
|
|
| Signals
|
Not Available
|
|
Transmembrane Regions
|
Not Available
|
| Protein Sequence
|
>Hydroxymethylglutaryl-CoA lyase, mitochondrial
MAAMRKALPRRLVGLASLRAVSTSSMGTLPKRVKIVEVGPRDGLQNEKNIVSTPVKIKLI
DMLSEAGLSVIETTSFVSPKWVPQMGDHTEVLKGIQKFPGINYPVLTPNLKGFEAAVAAG
AKEVVIFGAASELFTKKNINCSIEESFQRFDAILKAAQSANISVRGYVSCALGCPYEGKI
SPAKVAEVTKKFYSMGCYEISLGDTIGVGTPGIMKDMLSAVMQEVPLAALAVHCHDTYGQ
ALANTLMALQMGVSVVDSSVAGLGGCPYAQGASGNLATEDLVYMLEGLGIHTGVNLQKLL
EAGNFICQALNRKTSSKVAQATCKL
|
| External Links |
|---|
| GenBank ID Protein
| 14714839 |
| UniProtKB/Swiss-Prot ID
| P35914 |
| UniProtKB/Swiss-Prot Entry Name
| HMGCL_HUMAN |
| PDB IDs
|
|
| GenBank Gene ID
| BC010570 |
| GeneCard ID
| HMGCL |
| GenAtlas ID
| HMGCL |
| HGNC ID
| HGNC:5005 |
| References |
|---|
| General References
| Not Available |