Gene/Proteome Database (LMPD)
LMPD ID
LMP002304
Gene ID
Species
Mus musculus (Mouse)
Gene Name
3-hydroxy-3-methylglutaryl-Coenzyme A lyase
Gene Symbol
Synonyms
AW476067; HL
Alternate Names
hydroxymethylglutaryl-CoA lyase, mitochondrial; HMG-CoA lyase; 3-hydroxy-3-methylglutarate-CoA lyase
Chromosome
4
Map Location
4 D3|4 68.14 cM
EC Number
4.1.3.4
Proteins
| hydroxymethylglutaryl-CoA lyase, mitochondrial precursor | |
|---|---|
| Refseq ID | NP_032280 |
| Protein GI | 171543858 |
| UniProt ID | P38060 |
| mRNA ID | NM_008254 |
| Length | 325 |
| RefSeq Status | VALIDATED |
| MASVRKAFPRRLVGLTSLRAVSTSSMGTLPKQVKIVEVGPRDGLQNEKSIVPTPVKIRLIDMLSEAGLPVIEATSFVSPKWVPQMADHSDVLKGIQKFPGINYPVLTPNMKGFEEAVAAGAKEVSVFGAVSELFTRKNANCSIEESFQRFAGVMQAAQAASISVRGYVSCALGCPYEGKVSPAKVAEVAKKLYSMGCYEISLGDTIGVGTPGLMKDMLTAVMHEVPVTALAVHCHDTYGQALANTLVALQMGVSVVDSSVAGLGGCPYAKGASGNLATEDLVYMLNGLGIHTGVNLQKLLEAGDFICQALNRKTSSKVAQATCKL | |
| transit_peptide: 1..27 inference: non-experimental evidence, no additional details recorded note: Mitochondrion (By similarity); propagated from UniProtKB/Swiss-Prot (P38060.2) calculated_mol_wt: 2879 peptide sequence: MASVRKAFPRRLVGLTSLRAVSTSSMG mat_peptide: 28..325 product: Hydroxymethylglutaryl-CoA lyase, mitochondrial experiment: experimental evidence, no additional details recorded note: propagated from UniProtKB/Swiss-Prot (P38060.2) calculated_mol_wt: 31378 peptide sequence: TLPKQVKIVEVGPRDGLQNEKSIVPTPVKIRLIDMLSEAGLPVIEATSFVSPKWVPQMADHSDVLKGIQKFPGINYPVLTPNMKGFEEAVAAGAKEVSVFGAVSELFTRKNANCSIEESFQRFAGVMQAAQAASISVRGYVSCALGCPYEGKVSPAKVAEVAKKLYSMGCYEISLGDTIGVGTPGLMKDMLTAVMHEVPVTALAVHCHDTYGQALANTLVALQMGVSVVDSSVAGLGGCPYAKGASGNLATEDLVYMLNGLGIHTGVNLQKLLEAGDFICQALNRKTSSKVAQATCKL | |
Gene Information
Entrez Gene ID
Gene Name
3-hydroxy-3-methylglutaryl-Coenzyme A lyase
Gene Symbol
Species
Mus musculus
Gene Ontology (GO Annotations)
| GO ID | Source | Type | Description |
|---|---|---|---|
| GO:0005743 | IDA:MGI | C | mitochondrial inner membrane |
| GO:0005739 | IDA:MGI | C | mitochondrion |
| GO:0005777 | IEA:UniProtKB-KW | C | peroxisome |
| GO:0031406 | IEA:Ensembl | F | carboxylic acid binding |
| GO:0000062 | IEA:Ensembl | F | fatty-acyl-CoA binding |
| GO:0004419 | ISS:UniProtKB | F | hydroxymethylglutaryl-CoA lyase activity |
| GO:0000287 | IEA:Ensembl | F | magnesium ion binding |
| GO:0030145 | IEA:Ensembl | F | manganese ion binding |
| GO:0046872 | ISS:UniProtKB | F | metal ion binding |
| GO:0006637 | IEA:Ensembl | P | acyl-CoA metabolic process |
| GO:0046951 | ISS:UniProtKB | P | ketone body biosynthetic process |
| GO:0001889 | IEA:Ensembl | P | liver development |
| GO:0007005 | IMP:MGI | P | mitochondrion organization |
| GO:0051262 | IEA:Ensembl | P | protein tetramerization |
| GO:0070542 | IEA:Ensembl | P | response to fatty acid |
| GO:0007584 | IEA:Ensembl | P | response to nutrient |
| GO:0042594 | IEA:Ensembl | P | response to starvation |
KEGG Pathway Links
| KEGG Pathway ID | Description |
|---|---|
| mmu04146 | Peroxisome |
Domain Information
UniProt Annotations
Entry Information
Gene Name
3-hydroxy-3-methylglutaryl-Coenzyme A lyase
Protein Entry
HMGCL_MOUSE
UniProt ID
Species
Mouse
Comments
| Comment Type | Description |
|---|---|
| Catalytic Activity | (S)-3-hydroxy-3-methylglutaryl-CoA = acetyl- CoA + acetoacetate. {ECO:0000255|PROSITE-ProRule:PRU10115}. |
| Function | Key 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 (By similarity). {ECO:0000250}. |
| Pathway | Metabolic intermediate metabolism; (S)-3-hydroxy-3- methylglutaryl-CoA degradation; acetoacetate from (S)-3-hydroxy-3- methylglutaryl-CoA: step 1/1. |
| Similarity | Belongs to the HMG-CoA lyase family. {ECO:0000305}. |
| Subcellular Location | Mitochondrion matrix {ECO:0000269|PubMed:7527399}. Peroxisome {ECO:0000269|PubMed:7527399}. Note=Unprocessed form is peroxisomal. |
| Subunit | Homodimer; disulfide-linked. Can also form homotetramers. {ECO:0000250}. |
Identical and Related Proteins
Unique RefSeq proteins for LMP002304 (as displayed in Record Overview)
| Protein GI | Database | Accession | Length | Protein Name |
|---|---|---|---|---|
| 171543858 | RefSeq | NP_032280 | 325 | hydroxymethylglutaryl-CoA lyase, mitochondrial precursor |
Identical Sequences to LMP002304 proteins
| Reference | Database | Accession | Length | Protein Name |
|---|---|---|---|---|
| GI:171543858 | DBBJ | BAE32329.1 | 325 | unnamed protein product [Mus musculus] |
| GI:171543858 | DBBJ | BAE20956.1 | 325 | unnamed protein product [Mus musculus] |
| GI:171543858 | DBBJ | BAE26328.1 | 325 | unnamed protein product [Mus musculus] |
| GI:171543858 | GenBank | AAH25440.1 | 325 | Hmgcl protein [Mus musculus] |
| GI:171543858 | GenBank | EDL29963.1 | 325 | 3-hydroxy-3-methylglutaryl-Coenzyme A lyase [Mus musculus] |
| GI:171543858 | SwissProt | P38060.2 | 325 | RecName: Full=Hydroxymethylglutaryl-CoA lyase, mitochondrial; Short=HL; Short=HMG-CoA lyase; AltName: Full=3-hydroxy-3-methylglutarate-CoA lyase; Flags: Precursor [Mus musculus] |
Related Sequences to LMP002304 proteins
| Reference | Database | Accession | Length | Protein Name |
|---|---|---|---|---|
| GI:171543858 | GenBank | AAB03107.1 | 325 | 3-hydroxy-3-methylglutaryl-CoA lyase [Mus musculus] |
| GI:171543858 | GenBank | AEQ84587.1 | 325 | Sequence 81 from patent US 8039587 |
| GI:171543858 | GenBank | AEU53450.1 | 325 | Sequence 81 from patent US 8062891 |
| GI:171543858 | GenBank | AGU32394.1 | 325 | Sequence 81 from patent US 8507277 |
| GI:171543858 | GenBank | AGX48298.1 | 325 | Sequence 81 from patent US 8541550 |
| GI:171543858 | PIR | - | 325 | 3-Hydroxy-3-methylglutaryl coenzyme A lyase - mouse [Mus sp.] |