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Overview

Uniprot IDA0A8I5ZL37
Protein NamePropionyl-CoA carboxylase alpha chain, mitochondrial
Gene NamePcca
OrganismRattus norvegicus

Kla Sites from experimental identification

Position Flanking peptide
176 KLLAKRAKVNTIPGF

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. 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. Propionyl-CoA carboxylase also significantly acts on butyryl-CoA/butanoyl-CoA, which is converted to ethylmalonyl-CoA/(2S)-ethylmalonyl-CoA. Other alternative minor substrates include (2E)-butenoyl-CoA/crotonoyl-CoA

Protein Sequence

10 MAGLWVRTVA 20 LLAARRHWRR 30 SSQQLLWTLK 40 RAPVYSQQCL 50 VVSRSLSSVE 60 YEPKEKVSIL 70 KILSIVIKTC 80 RKMGIRTVAI 90 HSDVDASSVH 100 VKMADEAVCV 110 GPAPTSKSYL 120 NMDAIMEAIK 130 KTGAQAVHPG 140 YGFLSENKEF 150 AKCLAAEDVT 160 FIGPDTHAIQ 170 AMGDKIESKL 180 LAKRAKVNTI 190 PGFDGVVKDA 200 DEAVRIAREI 210 GYPVMIKASA 220 GGGGKGMRIA 230 WDDEETRDGF 240 RFSSQEAASS 250 FGDDRLLIEK 260 FIDNPRHIEI 270 QVLGDKHGNA 280 LWLNERECSI 290 QRRNQKVVEE 300 APSIFLDPET 310 RRAMGEQAVA 320 LAKAVKYSSA 330 GTVEFLVDSQ 340 KNFYFLEMNT 350 RLQVEHPVTE 360 CITGLDLVQE 370 MILVAKGYPL 380 RHKQEDIPIS 390 GWAVECRVYA 400 EDPYKSFGLP 410 SIGRLSQYQE 420 PIHLPGVRVD 430 SGIQPGSDIS 440 IYYDPMISKL 450 VTYGSDRAEA 460 LKRMEDALDS 470 YVIRGVTHNI 480 PLLREVIINT 490 RFVKGDISTK 500 FLSDVYPDGF 510 KGHMLTPSER 520 DQLLAIASSL 530 FVASQLRAQR 540 FQEHSRVPVI 550 RPDVAKWELS 560 VKLHDEDHTV 570 VASNNGPTFN 580 VEVDGSKLNV 590 TSTWNLASPL 600 LSVNVDGTQR 610 TVQCLSRDAG 620 GNMSIQFLGT 630 VYKVHILTKL 640 AAELNKFMLE 650 KVPKDTSSTL 660 CSPMPGVVVA 670 VSVKPGDMVA 680 EGQEICVIEA 690 MKMQNSMTAG 700 KMGKVKLVHC 710 KAGDTVGEGD LLVELE

Gene Ontology

Classification GO ID Description
Cellular Component GO:0005759 mitochondrial matrix
Molecular Function GO:0005524 ATP binding
Molecular Function GO:0046872 metal ion binding
Molecular Function GO:0004658 propionyl-CoA carboxylase activity
Biological Process GO:0019752 carboxylic acid metabolic process
Biological Process GO:0016042 lipid catabolic process

Reference

[1] Chen Y, Sun W, Sun Z, Zhao H, Wu T et al.. Effect of electroacupuncture on hippocampal protein lactylation in a rat model of vascular dementia.. Front Neurol 16:1629474. 2025. PMID: 40963935.