| URN | urn:agi-llid:3155 |
|---|---|
| Connectivity | 10 |
| Name | Hmgcl |
| Description | 3-hydroxymethyl-3-methylglutaryl-Coenzyme A lyase |
| Notes | an enzyme of the ketogenic 3-hydroxy-3-methylglutaryl-CoA cycle [RGD] |
| Pathway | ketone bodies metabolic pathway |
|---|
| Ariadne Ontology | Branced aminoacid metabolism |
|---|
| GO Molecular Function | catalytic activity |
|---|---|
| carboxylic acid binding | |
| acyl-CoA binding | |
| metal ion binding | |
| lyase activity | |
| hydroxymethylglutaryl-CoA lyase activity |
| GO Cellular Component | mitochondrion |
|---|---|
| mitochondrial matrix | |
| mitochondrial inner membrane |
| GO Biological Process | metabolic process |
|---|---|
| ketone body biosynthetic process | |
| acyl-CoA metabolic process |
| Group | Branced aminoacid metabolism |
|---|---|
| mitochondrion | |
| mitochondrial matrix | |
| mitochondrial inner membrane | |
| catalytic activity | |
| carboxylic acid binding | |
| acyl-CoA binding | |
| metal ion binding | |
| lyase activity | |
| hydroxymethylglutaryl-CoA lyase activity | |
| metabolic process | |
| ketone body biosynthetic process | |
| acyl-CoA metabolic process |
| Hugo ID | 5005 |
|---|
| Human chromosome position | 1p36.1-p35 |
|---|
| LocusLink ID | 3155 |
|---|---|
| 15356 | |
| 79238 |
| Alias | HL |
|---|---|
| 3-hydroxy-3-methylglutaryl CoA lyase | |
| OTTHUMP00000044830 | |
| hydroxymethylglutaricaciduria | |
| AW476067 | |
| RP23-161N17.7 | |
| OTTMUSP00000010411 | |
| 3-hydroxy-3-methylglutaryl-Coenzyme A lyase | |
| hydroxymethylglutaricaciduria protein | |
| Hydroxymethylglutaryl-CoA lyase | |
| Hydroxymethylglutaryl-CoA lyase, mitochondrial precursor | |
| mitochondrial Hydroxymethylglutaryl-CoA lyase precursor | |
| 3-hydroxy-3-methylglutarate-CoA lyase | |
| 3-hydroxy-3-methylglutaryl-Coenzyme A lyase (hydroxymethylglutaricaciduria) | |
| HMGCL | |
| hydroxymethylglutaricaciduria gene | |
| HMG-CoA lyase | |
| 3-hydroxymethyl-3-methylglutaryl-Coenzyme A lyase | |
| hydroxymethylglutaric aciduria gene | |
| hydroxymethylglutaric aciduria protein | |
| 3-hydroxymethyl-3-methylglutaryl-Coenzyme A lyase (hydroxymethylglutaricaciduria) | |
| LOC230831 |
| Organism | Homo sapiens |
|---|---|
| Mus musculus | |
| Rattus norvegicus |
| OMIM ID | 246450 |
|---|
| Swiss-Prot Accession | P35914 |
|---|---|
| Q6IBC0 | |
| Q8QZS6 | |
| P38060 | |
| Q3UMP2 | |
| P97519 |
| Unigene ID | Hs.533444 |
|---|---|
| Mm.439940 | |
| Rn.12297 |
| KEGG ID | hsa:3155 |
|---|---|
| mmu:15356 | |
| rno:79238 |
| EC Number | 4.1.3.4 |
|---|
| Swiss-Prot ID | HMGCL_HUMAN |
|---|---|
| HMGCL_RAT | |
| Q6IBC0_HUMAN | |
| Q8QZS6_MOUSE |
| Cell Localization | Cytoplasm |
|---|
| GO ID | 0004419 |
|---|---|
| 0016829 | |
| 0046872 | |
| 0008152 | |
| 0005743 | |
| 0005759 | |
| 0005739 | |
| 0003824 | |
| 0000062 | |
| 0031406 | |
| 0006637 | |
| 0046951 |
| IPI ID | IPI00293564 |
|---|---|
| IPI00514742 | |
| IPI00127625 | |
| IPI00379694 | |
| IPI00205374 |
| Mouse chromosome position | 4 65.6 cM |
|---|
| Homologene ID | 159 |
|---|
| MGI ID | 96158 |
|---|
| Rat chromosome position | 5q36 |
|---|
| RGD ID | 620554 |
|---|