Protein Name:
Propionyl-CoA carboxylase alpha chain, mitochondrial
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UniprotKB/SwissProt ID: P05165 (P05165)
Gene Name:
PCCA
Organism: Homo sapiens (Human)
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 (PubMed:6765947, PubMed:8434582). Propionyl-CoA carboxylase catalyzes the carboxylation of propionyl-CoA/propanoyl-CoA to D-methylmalonyl-CoA/(S)-methylmalonyl-CoA (PubMed:10101253, PubMed:6765947, PubMed:8434582). 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 at a much lower rate (PubMed:6765947). 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 P05165
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| No. |
Position |
S-nitrosylated Peptide |
Secondary Structure of S-nitrosylated Peptide |
Solvent Accessibility of nitrosylated Site |
PubMed ID |
| 1 |
111 |
VHVKMADEAV C VGPAPTSKSY |
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| 2 |
155 |
FLSENKEFAR C LAAEDVVFIG |
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37478858
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| 3 |
44 |
LKHVLYYSRQ C LMVSRNLGSV |
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| 4 |
712 |
GKTGTVKSVH C QAGDTVGEGD |
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