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Protein

Propionyl-CoA carboxylase alpha chain, mitochondrial

Gene

Pcca

Organism
Mus musculus (Mouse)
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 sitei173ATPBy similarity1
Binding sitei257ATPBy similarity1
Binding sitei292ATPBy similarity1
Active sitei349By similarity1

GO - Molecular functioni

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

Keywordsi

Molecular functionLigase
LigandATP-binding, Biotin, Nucleotide-binding

Enzyme and pathway databases

ReactomeiR-MMU-196780 Biotin transport and metabolism
R-MMU-71032 Propionyl-CoA catabolism
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
OrganismiMus musculus (Mouse)
Taxonomic identifieri10090 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresGliresRodentiaMyomorphaMuroideaMuridaeMurinaeMusMus
Proteomesi

Organism-specific databases

MGIiMGI:97499 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 – 48MitochondrionBy similarityAdd BLAST48
ChainiPRO_000000283849 – 724Propionyl-CoA carboxylase alpha chain, mitochondrialAdd BLAST676

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Modified residuei61N6-acetyllysine; alternateCombined sources1
Modified residuei61N6-succinyllysine; alternateCombined sources1
Modified residuei115N6-succinyllysineCombined sources1
Modified residuei146N6-acetyllysine; alternateCombined sources1
Modified residuei146N6-succinyllysine; alternateCombined sources1
Modified residuei150N6-acetyllysineCombined sources1
Modified residuei184N6-succinyllysineCombined sources1
Modified residuei196N6-acetyllysine; alternateCombined sources1
Modified residuei196N6-succinyllysine; alternateCombined sources1
Modified residuei248PhosphoserineCombined sources1
Modified residuei258N6-succinyllysineCombined sources1
Modified residuei324N6-acetyllysine; alternateCombined sources1
Modified residuei324N6-succinyllysine; alternateCombined sources1
Modified residuei381N6-succinyllysineCombined sources1
Modified residuei403N6-succinyllysineCombined sources1
Modified residuei492N6-acetyllysineCombined sources1
Modified residuei498N6-succinyllysineCombined sources1
Modified residuei509N6-succinyllysineCombined sources1
Modified residuei554N6-succinyllysineCombined sources1
Modified residuei644N6-succinyllysineCombined sources1
Modified residuei690N6-biotinyllysinePROSITE-ProRule annotationBy similarity1

Post-translational modificationi

Acetylated.1 Publication

Keywords - PTMi

Acetylation, Phosphoprotein

Proteomic databases

EPDiQ91ZA3
MaxQBiQ91ZA3
PaxDbiQ91ZA3
PRIDEiQ91ZA3

2D gel databases

REPRODUCTION-2DPAGEiQ91ZA3

PTM databases

iPTMnetiQ91ZA3
PhosphoSitePlusiQ91ZA3
SwissPalmiQ91ZA3

Expressioni

Gene expression databases

BgeeiENSMUSG00000041650
ExpressionAtlasiQ91ZA3 baseline and differential
GenevisibleiQ91ZA3 MM

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 (PubMed:23438705). Interacts with SIRT3 and SIRT5 (PubMed:23438705).Curated1 Publication

GO - Molecular functioni

Protein-protein interaction databases

BioGridi225934, 1 interactor
IntActiQ91ZA3, 4 interactors
MINTiQ91ZA3
STRINGi10090.ENSMUSP00000038763

Structurei

3D structure databases

ProteinModelPortaliQ91ZA3
SMRiQ91ZA3
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Domains and Repeats

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Domaini58 – 505Biotin carboxylationAdd BLAST448
Domaini177 – 374ATP-graspPROSITE-ProRule annotationAdd BLAST198
Domaini645 – 724Biotinyl-bindingPROSITE-ProRule annotationAdd BLAST80

Keywords - Domaini

Transit peptide

Phylogenomic databases

eggNOGiKOG0238 Eukaryota
COG4770 LUCA
GeneTreeiENSGT00550000074675
HOGENOMiHOG000008989
HOVERGENiHBG000555
InParanoidiQ91ZA3
KOiK01965
OMAiGGNMSIQ
OrthoDBiEOG091G06RG
PhylomeDBiQ91ZA3
TreeFamiTF354220

Family and domain databases

InterProiView protein in InterPro
IPR011761 ATP-grasp
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.

Q91ZA3-1 [UniParc]FASTAAdd to basket

« Hide

        10         20         30         40         50
MAGQWVRTVA LLAARRHWRR SSQQQLLGTL KHAPVYSYQC LVVSRSLSSV
60 70 80 90 100
EYEPKEKTFD KILIANRGEI ACRVIKTCKK MGIKTVAIHS DVDASSVHVK
110 120 130 140 150
MADEAVCVGP APTSKSYLNM DAIMEAIKKT RAQAVHPGYG FLSENKEFAK
160 170 180 190 200
RLAAEDVTFI GPDTHAIQAM GDKIESKLLA KRAKVNTIPG FDGVVKDADE
210 220 230 240 250
AVRIAREIGY PVMIKASAGG GGKGMRIAWD DEETRDGFRF SSQEAASSFG
260 270 280 290 300
DDRLLIEKFI DNPRHIEIQV LGDKHGNALW LNERECSIQR RNQKVVEEAP
310 320 330 340 350
SIFLDPETRQ AMGEQAVALA KAVKYSSAGT VEFLVDSQKN FYFLEMNTRL
360 370 380 390 400
QVEHPVTECI TGLDLVQEMI LVAKGYPLRH KQEDIPISGW AVECRVYAED
410 420 430 440 450
PYKSFGLPSI GRLSQYQEPI HLPGVRVDSG IQPGSDISIY YDPMISKLVT
460 470 480 490 500
YGSDRAEALK RMEDALDNYV IRGVTHNIPL LREVIINTRF VKGDISTKFL
510 520 530 540 550
SDVYPDGFKG HTLTLSERNQ LLAIASSVFV ASQLRAQRFQ EHSRVPVIRP
560 570 580 590 600
DVAKWELSVK LHDEDHTVVA SNNGPAFTVE VDGSKLNVTS TWNLASPLLS
610 620 630 640 650
VNVDGTQRTV QCLSREAGGN MSIQFLGTVY KVHILTKLAA ELNKFMLEKV
660 670 680 690 700
PKDTSSTLCS PMPGVVVAVS VKPGDMVAEG QEICVIEAMK MQNSMTAGKM
710 720
GKVKLVHCKA GDTVGEGDLL VELE
Length:724
Mass (Da):79,922
Last modified:May 24, 2005 - v2
Checksum:i249189EDF9F99274
GO

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sequence conflicti497T → A in AAL02364 (PubMed:11461925).Curated1
Sequence conflicti542H → R in AAH06915 (PubMed:15489334).Curated1
Sequence conflicti555 – 561WELSVKL → VGALGKV in AAL02364 (PubMed:11461925).Curated7

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AY046947 mRNA Translation: AAL02364.1
BC006915 mRNA Translation: AAH06915.1
BC049802 mRNA Translation: AAH49802.1
CCDSiCCDS27350.1
RefSeqiNP_659093.2, NM_144844.2
UniGeneiMm.23876

Genome annotation databases

EnsembliENSMUST00000038374; ENSMUSP00000038763; ENSMUSG00000041650
GeneIDi110821
KEGGimmu:110821
UCSCiuc007vbe.2 mouse

Similar proteinsi

Entry informationi

Entry nameiPCCA_MOUSE
AccessioniPrimary (citable) accession number: Q91ZA3
Secondary accession number(s): Q80VU5, Q922N3
Entry historyiIntegrated into UniProtKB/Swiss-Prot: May 24, 2005
Last sequence update: May 24, 2005
Last modified: May 23, 2018
This is version 133 of the entry and version 2 of the sequence. See complete history.
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program

Miscellaneousi

Keywords - Technical termi

Complete proteome, Reference proteome

Documents

  1. MGD cross-references
    Mouse Genome Database (MGD) cross-references in UniProtKB/Swiss-Prot
  2. PATHWAY comments
    Index of metabolic and biosynthesis pathways

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