Overview of BCAA catabolic pathway
Overview of BCAA catabolic pathway. The BCAAs undergo transamination that is catalyzed by the branched-chain aminotransferase (BCAT) and requires α- ketoglutarate, leading to the production of the α-ketoacids KIC, KMV, and KIV. These intermediates then undergo oxidative decarboxylation, catalyzed by the BCKAD complex.Abbreviations: KIC, α-ketoisocaproic acid; KIV, α-ketoisovaleric acid; KMV, α-keto-β-methylvaleric acid; Glu, glutamate; BCAAs, branched-chain amino acids; BCKAD, branched-chain α-ketoacid dehydrogenase.
Publication
Maple syrup urine disease: mechanisms and management. (2017) Patrick R Blackburn, et al. Appl Clin Genet. 2017;10:57-66. Figure: F1.| Organism | Group | Word | Match | Source | NCBI Symbol | NCBI ID |
|---|---|---|---|---|---|---|
| Homo sapiens | Primates | BCKDK | BCKDK | ncbigene_symbol | BCKDK | 10295 |
| Homo sapiens | Primates | E1a | E1A | ncbigene_synonym | PDHA1 | 5160 |
| Homo sapiens | Primates | E1a | E1A | ncbigene_synonym | DHTKD1 | 55526 |
| Homo sapiens | Primates | PPM1K | PPM1K | ncbigene_symbol | PPM1K | 152926 |
| Homo sapiens | Primates | E1B | E1B | ncbigene_synonym | BCKDHB | 594 |
| Homo sapiens | Primates | E1B | E1B | ncbigene_synonym | PDHB | 5162 |
| Homo sapiens | Primates | E1B | E1B | ncbigene_synonym | SNORA73A | 6080 |
| Word | Match | MeSH | Name | ChEBI |
|---|---|---|---|---|
| Succinyl-CoA | NA | mesh:C012046 | ||
| succinyl-CoA | NA | mesh:C012046 | ||
| acetoacetate | NA | mesh:D000090 | ||
| Isoleucine | Isoleucine | mesh:D007532 | L-isoleucine | chebi:17191 |
| Leucine | Leucine | mesh:D007930 | L-leucine | chebi:15603 |
| Valine | Valine | mesh:D014633 | L-valine | chebi:16414 |
| Glu | NA | mesh:D018698 |
Disease mentions
| Word | Match | MeSH | Name | DOID |
|---|