Major pathway of the conversion of propionyl-CoA into succinyl-CoA in human cells
Major pathway of the conversion of propionyl-CoA into succinyl-CoA in human cells. The biotin-dependent enzyme, propionyl-CoA carboxylase (PCCA, PCCB) converts propionyl-CoA into D-methylmalonyl-CoA which is then racemized into L-methylmalonyl-CoA and isomerized into succinyl-CoA, a Kreb cycle intermediate. The L-methylmalonyl-CoA mutase (MUT) reaction requires adenosylcobalamin. The class and genes associated with methylmalonic acidemia are mut (MUT), cblA (MMAA), cblB (MMAB), cblD variant 2 (unknown gene(s)), cblC (MMAHC), cblF (unknown gene(s)). Question marks indicate unknown genes or poorly defined cellular processes.
Publication
Propionyl-CoA and Adenosylcobalamin Metabolism in C. elegans : Evidence for a Role of Methylmalonyl-CoA Epimerase in Intermediary Metabolism. () Randy J. Chandler, et al. Mol Genet Metab. ;89(1-2):64-73. Figure: F1.| Organism | Group | Word | Match | Source | NCBI Symbol | NCBI ID |
|---|---|---|---|---|---|---|
| Homo sapiens | Primates | MMAA | MMAA | ncbigene_symbol | MMAA | 166785 |
| Homo sapiens | Primates | MMAA | MMAA | ncbigene_synonym | SLC7A5P2 | 387254 |
| Homo sapiens | Primates | (MUT) | MUT | ncbigene_synonym | MMUT | 4594 |
| Homo sapiens | Primates | (MCEE) | MCEE | ncbigene_symbol | MCEE | 84693 |
| Homo sapiens | Primates | (MMAB) | MMAB | ncbigene_symbol | MMAB | 326625 |
| Homo sapiens | Primates | (PCCA,PCCB) | PCCB | ncbigene_symbol | PCCB | 5096 |
| Homo sapiens | Primates | (PCCA,PCCB) | PCCA | ncbigene_symbol | PCCA | 5095 |
| Homo sapiens | Primates | Cbl(?) | CBL | ncbigene_symbol | CBL | 867 |
| Homo sapiens | Primates | Cbl(?) | CB1 | ncbigene_synonym | CNR1 | 1268 |
| Homo sapiens | Primates | MMACHC | MMACHC | ncbigene_symbol | MMACHC | 25974 |
| Danio rerio | Vertebrates | MMAA | MMAA | ncbigene_symbol | mmaa | 564760 |
| Danio rerio | Vertebrates | (MUT) | MUT | ncbigene_synonym | mmut | 569581 |
| Danio rerio | Vertebrates | (MCEE) | MCEE | ncbigene_symbol | mcee | 553804 |
| Danio rerio | Vertebrates | (MMAB) | MMAB | ncbigene_symbol | mmab | 557077 |
| Danio rerio | Vertebrates | Cbl(1?) | CB11 | ncbigene_synonym | sb:cb11 | 321034 |
| Danio rerio | Vertebrates | (PCCA,PCCB) | PCCB | ncbigene_symbol | pccb | 405861 |
| Danio rerio | Vertebrates | (PCCA,PCCB) | PCCA | ncbigene_symbol | pcca | 437019 |
| Danio rerio | Vertebrates | (PCCA,PCCB) | PCCA | ncbigene_synonym | zc3h13 | 560041 |
| Danio rerio | Vertebrates | Cbl(?) | CBL | ncbigene_symbol | cbl | 492364 |
| Danio rerio | Vertebrates | Cbl(?) | CBL | ncbigene_synonym | cblb | 556277 |
| Danio rerio | Vertebrates | Cbl(?) | CB1 | ncbigene_synonym | kif11 | 195818 |
| Danio rerio | Vertebrates | Cbl(?) | CB1 | ncbigene_synonym | cnr1 | 404209 |
| Danio rerio | Vertebrates | MMACHC | MMACHC | ncbigene_symbol | mmachc | 555267 |
| Word | Match | MeSH | Name | ChEBI |
|---|---|---|---|---|
| Adenosylcobalamin | NA | mesh:C000913 | ||
| Propionyl-CoA | propionyl-CoA | mesh:C009061 | propionyl-coenzyme A | chebi:15539 |
| Succinyl-CoA | NA | mesh:C012046 | ||
| Biotin | Biotin | mesh:D001710 | biotin | chebi:15956 |
Disease mentions
| Word | Match | MeSH | Name | DOID |
|---|