Protein Name:
Propionyl-CoA carboxylase alpha chain, mitochondrial
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UniprotKB/SwissProt ID: Q91ZA3 (Q91ZA3)
Gene Name:
Pcca
Organism: Mus musculus (Mouse)
Function: This is one of the 2 subunits of the biotin-dependent propionyl-CoA carboxylase (PCC), a mitochondrial enzyme involved in the catabolism of odd chain fatty acids, branched-chain amino acids isoleucine, threonine, methionine, and valine and other metabolites. Propionyl-CoA carboxylase catalyzes the carboxylation of propionyl-CoA/propanoyl-CoA to D-methylmalonyl-CoA/(S)-methylmalonyl-CoA (By similarity). Within the holoenzyme, the alpha subunit catalyzes the ATP-dependent carboxylation of the biotin carried by the biotin carboxyl carrier (BCC) domain, while the beta subunit then transfers the carboxyl group from carboxylated biotin to propionyl-CoA (By similarity). Propionyl-CoA carboxylase also significantly acts on butyryl-CoA/butanoyl-CoA, which is converted to ethylmalonyl-CoA/(2S)-ethylmalonyl-CoA (By similarity). Other alternative minor substrates include (2E)-butenoyl-CoA/crotonoyl-CoA (By similarity)
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 Q91ZA3
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| No. |
Position |
S-nitrosylated Peptide |
Secondary Structure of S-nitrosylated Peptide |
Solvent Accessibility of nitrosylated Site |
PubMed ID |
| 1 |
107 |
VHVKMADEAV C VGPAPTSKSY |
CCCHHHHHHH H HHHHHHHHHC |
3.64% |
21278135
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| 2 |
359 |
RLQVEHPVTE C ITGLDLVQEM |
CCCHHHHHHH H HHHHHHHHHC |
2.17% |
21278135
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| 3 |
394 |
DIPISGWAVE C RVYAEDPYKS |
CCCHHHHHHH H HHHHHHHHHC |
2.61% |
21278135
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