Protein Name:
2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial
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UniprotKB/SwissProt ID: P50136 (P50136)
Gene Name:
Bckdha
Organism: Mus musculus (Mouse)
Function: Together with BCKDHB forms the heterotetrameric E1 subunit of the mitochondrial branched-chain alpha-ketoacid dehydrogenase (BCKD) complex. The BCKD complex catalyzes the multi-step oxidative decarboxylation of alpha-ketoacids derived from the branched-chain amino-acids valine, leucine and isoleucine producing CO2 and acyl-CoA which is subsequently utilized to produce energy. The E1 subunit catalyzes the first step with the decarboxylation of the alpha-ketoacid forming an enzyme-product intermediate. A reductive acylation mediated by the lipoylamide cofactor of E2 extracts the acyl group from the E1 active site for the next step of the reaction
Other Modifications: View all modification sites in dbPTM
Protein Subcellular Localization: Mitochondrion matrix
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Graphical Visualization of S-nitrosylation Sites:
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The S-nitrosylation sites of P50136
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| No. |
Position |
S-nitrosylated Peptide |
Secondary Structure of S-nitrosylated Peptide |
Solvent Accessibility of nitrosylated Site |
PubMed ID |
| 1 |
175 |
DYPLELFMSQ C YGNVNDPGKG |
CCHHHHCCCC H HHHHHHHHHH |
3.54% |
21278135
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| 2 |
194 |
KGRQMPVHYG C KERHFVTISS |
CCHHHHCCCC H HHHHHHHHHH |
3.05% |
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