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Overview

Uniprot IDA0A0G2K5P9
Protein NamePropionyl-CoA carboxylase alpha chain, mitochondrial
Gene NamePcca
OrganismRattus norvegicus

Kla Sites from experimental identification

Position Flanking peptide
191 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 VSKAGLAWRA 50 PVYSQQCLVV 60 SRSLSSVEYE 70 PKEKTFDKIL 80 IANRGEIACR 90 VIKTCRKMGI 100 RTVAIHSDVD 110 ASSVHVKMAD 120 EAVCVGPAPT 130 SKSYLNMDAI 140 MEAIKKTGAQ 150 AVHPGYGFLS 160 ENKEFAKCLA 170 AEDVTFIGPD 180 THAIQAMGDK 190 IESKLLAKRA 200 KVNTIPGFDG 210 VVKDADEAVR 220 IAREIGYPVM 230 IKASAGGGGK 240 GMRIAWDDEE 250 TRDGFRFSSQ 260 EAASSFGDDR 270 LLIEKFIDNP 280 RHIEIQVLGD 290 KHGNALWLNE 300 RECSIQRRNQ 310 KVVEEAPSIF 320 LDPETRRAMG 330 EQAVALAKAV 340 KYSSAGTVEF 350 LVDSQKNFYF 360 LEMNTRLQVE 370 HPVTECITGL 380 DLVQEMILVA 390 KGYPLRHKQE 400 DIPISGWAVE 410 CRVYAEDPYK 420 SFGLPSIGRL 430 SQYQEPIHLP 440 GVTLDLCHTF 450 MLENLVTYGS 460 DRAEALKRME 470 DALDSYVIRG 480 VTHNIPLLRE 490 VIINTRFVKG 500 DISTKFLSDV 510 YPDGFKGHML 520 TPSERDQLLA 530 IASSLFVASQ 540 LRAQRFQEHS 550 RVPVIRPDVA 560 KWELSVKLHD 570 EDHTVVASNN 580 GPTFNVEVDG 590 SKLNVTSTWN 600 LASPLLSVNV 610 DGTQRTVQCL 620 SRDAGGNMSI 630 QFLGTVYKVH 640 ILTKLAAELN 650 KFMLEKVPKD 660 TSSTLCSPMP 670 GVVVAVSVKP 680 GDMVAEGQEI 690 CVIEAMKMQN 700 SMTAGKMGKV 710 KLVHCKAGDT 720 VGEGDLLVEL E

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.