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Protein

Propionyl-CoA carboxylase alpha chain, mitochondrial

Gene

Pcca

Organism
Rattus norvegicus (Rat)
Status
Reviewed-Annotation score: -Experimental evidence at protein leveli

Functioni

Catalytic activityi

ATP + propanoyl-CoA + HCO3- = ADP + phosphate + (S)-methylmalonyl-CoA.

Cofactori

Pathwayi: propanoyl-CoA degradation

This protein is involved in step 1 of the subpathway that synthesizes succinyl-CoA from propanoyl-CoA.
Proteins known to be involved in the 3 steps of the subpathway in this organism are:
  1. Propionyl-CoA carboxylase alpha chain, mitochondrial (Pcca), Propionyl-CoA carboxylase beta chain, mitochondrial (Pccb)
  2. no protein annotated in this organism
  3. no protein annotated in this organism
This subpathway is part of the pathway propanoyl-CoA degradation, which is itself part of Metabolic intermediate metabolism.
View all proteins of this organism that are known to be involved in the subpathway that synthesizes succinyl-CoA from propanoyl-CoA, the pathway propanoyl-CoA degradation and in Metabolic intermediate metabolism.

Sites

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Binding sitei186ATPBy similarity1
Binding sitei270ATPBy similarity1
Binding sitei305ATPBy similarity1
Active sitei362By similarity1

GO - Molecular functioni

  • ATP binding Source: UniProtKB-KW
  • metal ion binding Source: InterPro
  • propionyl-CoA carboxylase activity Source: RGD

GO - Biological processi

  • cellular amino acid catabolic process Source: RGD
  • fatty acid catabolic process Source: RGD

Keywordsi

Molecular functionLigase
LigandATP-binding, Biotin, Nucleotide-binding

Enzyme and pathway databases

BioCyciMetaCyc:MONOMER-8606
BRENDAi6.4.1.3 5301
UniPathwayiUPA00945; UER00908

Names & Taxonomyi

Protein namesi
Recommended name:
Propionyl-CoA carboxylase alpha chain, mitochondrial (EC:6.4.1.3)
Short name:
PCCase subunit alpha
Alternative name(s):
Propanoyl-CoA:carbon dioxide ligase subunit alpha
Gene namesi
Name:Pcca
OrganismiRattus norvegicus (Rat)
Taxonomic identifieri10116 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresGliresRodentiaMyomorphaMuroideaMuridaeMurinaeRattus
Proteomesi
  • UP000002494 Componenti: Unplaced

Organism-specific databases

RGDi3264 Pcca

Subcellular locationi

Extracellular region or secreted Cytosol Plasma membrane Cytoskeleton Lysosome Endosome Peroxisome ER Golgi apparatus Nucleus Mitochondrion Manual annotation Automatic computational assertionGraphics by Christian Stolte; Source: COMPARTMENTS

Keywords - Cellular componenti

Mitochondrion

Pathology & Biotechi

Involvement in diseasei

Propionic acidemia due to recessively inherited deficiency of PCCase activity often causes life-threatening ketosis and acidosis.

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Transit peptidei1 – 61MitochondrionBy similarityAdd BLAST61
ChainiPRO_000000283962 – 737Propionyl-CoA carboxylase alpha chain, mitochondrialAdd BLAST676

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Modified residuei74N6-acetyllysine; alternateBy similarity1
Modified residuei74N6-succinyllysine; alternateBy similarity1
Modified residuei128N6-succinyllysineBy similarity1
Modified residuei159N6-acetyllysine; alternateBy similarity1
Modified residuei159N6-succinyllysine; alternateBy similarity1
Modified residuei163N6-acetyllysineBy similarity1
Modified residuei197N6-succinyllysineBy similarity1
Modified residuei209N6-acetyllysine; alternateBy similarity1
Modified residuei209N6-succinyllysine; alternateBy similarity1
Modified residuei261PhosphoserineCombined sources1
Modified residuei271N6-succinyllysineBy similarity1
Modified residuei337N6-acetyllysine; alternateBy similarity1
Modified residuei337N6-succinyllysine; alternateBy similarity1
Modified residuei394N6-succinyllysineBy similarity1
Modified residuei416N6-succinyllysineBy similarity1
Modified residuei505N6-acetyllysineBy similarity1
Modified residuei511N6-succinyllysineBy similarity1
Modified residuei522N6-succinyllysineBy similarity1
Modified residuei567N6-succinyllysineBy similarity1
Modified residuei657N6-succinyllysineBy similarity1
Modified residuei703N6-biotinyllysinePROSITE-ProRule annotationBy similarity1

Post-translational modificationi

Acetylated.By similarity

Keywords - PTMi

Acetylation, Phosphoprotein

Proteomic databases

PRIDEiP14882

PTM databases

iPTMnetiP14882
PhosphoSitePlusiP14882

Interactioni

Subunit structurei

Probably a dodecamer composed of six biotin-containing alpha subunits and six beta subunits (Probable). Interacts (via the biotin carboxylation domain) with SIRT4. Interacts with SIRT3 and SIRT5 (By similarity).By similarityCurated

Structurei

3D structure databases

ProteinModelPortaliP14882
SMRiP14882
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Domains and Repeats

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Domaini71 – 518Biotin carboxylationAdd BLAST448
Domaini190 – 387ATP-graspPROSITE-ProRule annotationAdd BLAST198
Domaini658 – 737Biotinyl-bindingPROSITE-ProRule annotationAdd BLAST80

Keywords - Domaini

Transit peptide

Phylogenomic databases

HOGENOMiHOG000008989
HOVERGENiHBG000555
InParanoidiP14882
KOiK01965
PhylomeDBiP14882

Family and domain databases

Gene3Di3.30.1490.20, 1 hit
InterProiView protein in InterPro
IPR011761 ATP-grasp
IPR013815 ATP_grasp_subdomain_1
IPR005481 BC-like_N
IPR001882 Biotin_BS
IPR011764 Biotin_carboxylation_dom
IPR005482 Biotin_COase_C
IPR000089 Biotin_lipoyl
IPR005479 CbamoylP_synth_lsu-like_ATP-bd
IPR016185 PreATP-grasp_dom_sf
IPR011054 Rudment_hybrid_motif
IPR011053 Single_hybrid_motif
PfamiView protein in Pfam
PF02785 Biotin_carb_C, 1 hit
PF00289 Biotin_carb_N, 1 hit
PF00364 Biotin_lipoyl, 1 hit
PF02786 CPSase_L_D2, 1 hit
SMARTiView protein in SMART
SM00878 Biotin_carb_C, 1 hit
SUPFAMiSSF51230 SSF51230, 1 hit
SSF51246 SSF51246, 1 hit
SSF52440 SSF52440, 1 hit
PROSITEiView protein in PROSITE
PS50975 ATP_GRASP, 1 hit
PS50979 BC, 1 hit
PS00188 BIOTIN, 1 hit
PS50968 BIOTINYL_LIPOYL, 1 hit
PS00866 CPSASE_1, 1 hit
PS00867 CPSASE_2, 1 hit

Sequencei

Sequence statusi: Complete.

Sequence processingi: The displayed sequence is further processed into a mature form.

P14882-1 [UniParc]FASTAAdd to basket

« Hide

        10         20         30         40         50
MAGLWVRTVA LLAARRHWRR SSQQLLWTLK RAPRSSQQLL WTLKRAPVYS
60 70 80 90 100
QQCLVVSRSL SSVEYEPKEK TFDKILIANR GEIACRVIKT CRKMGIRTVA
110 120 130 140 150
IHSDVDASSV HVKMADEAVC VGPAPTSKSY LNMDAIMEAI KKTGAQAVHP
160 170 180 190 200
GYGFLSENKE FAKCLAAEDV TFIGPDTHAI QAMGDKIESK LLAKRAKVNT
210 220 230 240 250
IPGFDGVLKD ADEAVRIARE IGYPVMIKAS AGGGGKGMRI PWDDEETRDG
260 270 280 290 300
FRFSSQEAAS SFGDDRLLIE KFIDNPRHIE IQVLGDKHGN ALWLNERECS
310 320 330 340 350
IQRRNQKVVE EAPSIFLDPE TRRAMGEQAV AWPKAVKYSS AGTVEFLVDS
360 370 380 390 400
QKNFYFLEMN TRLQVEHPVT ECITGLDLVQ EMILVAKGYP LRHKQEDIPI
410 420 430 440 450
SGWAVECRVY AEDPYKSFGL PSIGRLSQYQ EPIHLPGVRV DSGIQPGSDI
460 470 480 490 500
SIYHDPMISK LVTYGSDRAE ALKRMEDALD SYVIRGVTHN IPLLREVIIN
510 520 530 540 550
TRFVKGDIST KFLSDVYPDG FKGHMLTPSE RDQLLAIASS LFVASQLRAQ
560 570 580 590 600
RFQEHSRVPV IRPDVAKWEL SVKLHDEDHT VVASNNGPTF NVEVDGSKLN
610 620 630 640 650
VTSTWNLASP LLSVNVDGTQ RTVQCLSPDA GGNMSIQFLG TVYKVHILTK
660 670 680 690 700
LAAELNKFML EKVPKDTSSV LRSPKPGVVV AVSVKPGDMV AEGQEICVIE
710 720 730
AMKMQNSMTA GKMGKVKLVH CKAGDTVGEG DLLVELE
Length:737
Mass (Da):81,623
Last modified:November 30, 2010 - v3
Checksum:i58D13E5033A1D024
GO

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
CK228512 mRNA No translation available.
M22631 mRNA Translation: AAA88512.1 Sequence problems.
PIRiA34337
RefSeqiNP_062203.1, NM_019330.1
UniGeneiRn.6033

Genome annotation databases

GeneIDi687008
KEGGirno:687008

Similar proteinsi

Entry informationi

Entry nameiPCCA_RAT
AccessioniPrimary (citable) accession number: P14882
Entry historyiIntegrated into UniProtKB/Swiss-Prot: April 1, 1990
Last sequence update: November 30, 2010
Last modified: May 23, 2018
This is version 149 of the entry and version 3 of the sequence. See complete history.
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program

Miscellaneousi

Keywords - Technical termi

Complete proteome, Direct protein sequencing, Reference proteome

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