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Protein

Propionyl-CoA carboxylase alpha chain, mitochondrial

Gene

PCCA

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score: Annotation score: 5 out of 5-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)LengthDescriptionGraphical viewFeature identifierActions
Binding sitei177 – 1771ATPBy similarity
Binding sitei261 – 2611ATPBy similarity
Binding sitei296 – 2961ATPBy similarity
Active sitei353 – 3531By similarity

GO - Molecular functioni

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

GO - Biological processi

Complete GO annotation...

Keywords - Molecular functioni

Ligase

Keywords - Ligandi

ATP-binding, Biotin, Nucleotide-binding

Enzyme and pathway databases

BioCyciMetaCyc:ENSG00000175198-MONOMER.
RETL1328306-WGS:GSTH-2235-MONOMER.
ReactomeiR-HSA-196780. Biotin transport and metabolism.
R-HSA-3371599. Defective HLCS causes multiple carboxylase deficiency.
R-HSA-71032. Propionyl-CoA catabolism.
SABIO-RKP05165.
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
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 13

Organism-specific databases

HGNCiHGNC:8653. PCCA.

Subcellular locationi

GO - Cellular componenti

Complete GO annotation...

Keywords - Cellular componenti

Mitochondrion

Pathology & Biotechi

Involvement in diseasei

Propionic acidemia type I (PA-1)4 Publications
The disease is caused by mutations affecting the gene represented in this entry.
Disease descriptionLife-threatening disease characterized by episodic vomiting, lethargy and ketosis, neutropenia, periodic thrombocytopenia, hypogammaglobulinemia, developmental retardation, and intolerance to protein.
See also OMIM:606054
Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Natural varianti75 – 751A → P in PA-1. 1 Publication
Corresponds to variant rs794727479 [ dbSNP | Ensembl ].
VAR_009087
Natural varianti77 – 771R → W in PA-1. 2 Publications
Corresponds to variant rs141371306 [ dbSNP | Ensembl ].
VAR_009088
Natural varianti138 – 1381A → T in PA-1. 1 Publication
Corresponds to variant rs202247814 [ dbSNP | Ensembl ].
VAR_009089
Natural varianti164 – 1641I → T in PA-1. 1 Publication
Corresponds to variant rs202247815 [ dbSNP | Ensembl ].
VAR_009090
Natural varianti197 – 1971G → E in PA-1. 1 Publication
VAR_023843
Natural varianti229 – 2291M → K in PA-1. 1 Publication
VAR_009091
Natural varianti297 – 2971Q → R in PA-1. 1 Publication
VAR_009092
Natural varianti368 – 3681D → G in PA-1. 1 Publication
VAR_009093
Natural varianti373 – 3731M → K in PA-1; unstable protein. 1 Publication
Corresponds to variant rs121964958 [ dbSNP | Ensembl ].
VAR_009094
Natural varianti379 – 3791G → V in PA-1. 1 Publication
Corresponds to variant rs794727087 [ dbSNP | Ensembl ].
VAR_009095
Natural varianti398 – 3981C → R in PA-1. 1 Publication
VAR_023844
Natural varianti399 – 3991R → Q in PA-1. 1 Publication
VAR_009096
Natural varianti423 – 4231P → L in PA-1. 1 Publication
VAR_009097
Natural varianti532 – 5321Missing in PA-1. 1 Publication
VAR_023845
Natural varianti559 – 5591W → L in PA-1. 1 Publication
Corresponds to variant rs118169528 [ dbSNP | Ensembl ].
VAR_009099
Natural varianti631 – 6311G → R in PA-1. 1 Publication
Corresponds to variant rs796052018 [ dbSNP | Ensembl ].
VAR_009100
Natural varianti668 – 6681G → R in PA-1. 1 Publication
Corresponds to variant rs771438170 [ dbSNP | Ensembl ].
VAR_009101
Natural varianti712 – 7121Missing in PA-1. 1 Publication
VAR_009102

Keywords - Diseasei

Disease mutation

Organism-specific databases

MalaCardsiPCCA.
MIMi606054. phenotype.
Orphaneti35. Propionic acidemia.
PharmGKBiPA32992.

Chemistry

DrugBankiDB00121. Biotin.

Polymorphism and mutation databases

BioMutaiPCCA.
DMDMi308153661.

PTM / Processingi

Molecule processing

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Transit peptidei1 – 5252Mitochondrion1 PublicationAdd
BLAST
Chaini53 – 728676Propionyl-CoA carboxylase alpha chain, mitochondrialPRO_0000002837Add
BLAST

Amino acid modifications

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Modified residuei65 – 651N6-acetyllysine; alternateBy similarity
Modified residuei65 – 651N6-succinyllysine; alternateBy similarity
Modified residuei119 – 1191N6-succinyllysineBy similarity
Modified residuei150 – 1501N6-acetyllysine; alternateBy similarity
Modified residuei150 – 1501N6-succinyllysine; alternateBy similarity
Modified residuei200 – 2001N6-acetyllysine; alternateBy similarity
Modified residuei200 – 2001N6-succinyllysine; alternateBy similarity
Modified residuei252 – 2521PhosphoserineBy similarity
Modified residuei262 – 2621N6-succinyllysineBy similarity
Modified residuei328 – 3281N6-acetyllysine; alternateBy similarity
Modified residuei328 – 3281N6-succinyllysine; alternateBy similarity
Modified residuei385 – 3851N6-succinyllysineBy similarity
Modified residuei407 – 4071N6-succinyllysineBy similarity
Modified residuei496 – 4961N6-acetyllysineBy similarity
Modified residuei502 – 5021N6-succinyllysineBy similarity
Modified residuei513 – 5131N6-succinyllysineBy similarity
Modified residuei648 – 6481N6-succinyllysineBy similarity
Modified residuei694 – 6941N6-biotinyllysinePROSITE-ProRule annotationBy similarity

Keywords - PTMi

Acetylation, Phosphoprotein

Proteomic databases

EPDiP05165.
MaxQBiP05165.
PaxDbiP05165.
PeptideAtlasiP05165.
PRIDEiP05165.

PTM databases

iPTMnetiP05165.
PhosphoSiteiP05165.
SwissPalmiP05165.

Expressioni

Gene expression databases

BgeeiENSG00000175198.
CleanExiHS_PCCA.
ExpressionAtlasiP05165. baseline and differential.
GenevisibleiP05165. HS.

Organism-specific databases

HPAiHPA041716.
HPA047792.

Interactioni

Subunit structurei

Probably a dodecamer composed of six biotin-containing alpha subunits and six beta subunits.

GO - Molecular functioni

  • enzyme binding Source: UniProtKB

Protein-protein interaction databases

BioGridi111128. 21 interactions.
DIPiDIP-57493N.
IntActiP05165. 14 interactions.
STRINGi9606.ENSP00000365462.

Structurei

Secondary structure

1
728
Legend: HelixTurnBeta strand
Show more details
Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Beta strandi199 – 2013Combined sources
Helixi202 – 21211Combined sources
Beta strandi214 – 2207Combined sources
Turni226 – 2283Combined sources
Beta strandi230 – 2345Combined sources
Helixi235 – 25218Combined sources
Beta strandi258 – 2625Combined sources
Beta strandi265 – 2684Combined sources
Beta strandi665 – 6673Combined sources
Beta strandi669 – 6735Combined sources
Beta strandi688 – 6914Combined sources

3D structure databases

Select the link destinations:
PDBei
RCSB PDBi
PDBji
Links Updated
EntryMethodResolution (Å)ChainPositionsPDBsum
2CQYNMR-A176-270[»]
2JKUX-ray1.50A658-728[»]
ProteinModelPortaliP05165.
SMRiP05165. Positions 63-728.
ModBaseiSearch...
MobiDBiSearch...

Miscellaneous databases

EvolutionaryTraceiP05165.

Family & Domainsi

Domains and Repeats

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Domaini62 – 509448Biotin carboxylationAdd
BLAST
Domaini181 – 378198ATP-graspPROSITE-ProRule annotationAdd
BLAST
Domaini653 – 72876Biotinyl-bindingPROSITE-ProRule annotationAdd
BLAST

Sequence similaritiesi

Contains 1 ATP-grasp domain.PROSITE-ProRule annotation
Contains 1 biotin carboxylation domain.Curated
Contains 1 biotinyl-binding domain.PROSITE-ProRule annotationCurated

Keywords - Domaini

Transit peptide

Phylogenomic databases

eggNOGiKOG0238. Eukaryota.
COG4770. LUCA.
GeneTreeiENSGT00550000074675.
HOGENOMiHOG000008989.
HOVERGENiHBG000555.
InParanoidiP05165.
KOiK01965.
OMAiDIKLKGW.
OrthoDBiEOG091G06RG.
PhylomeDBiP05165.
TreeFamiTF354220.

Family and domain databases

Gene3Di3.30.1490.20. 1 hit.
3.30.470.20. 1 hit.
3.40.50.20. 1 hit.
InterProiIPR011761. ATP-grasp.
IPR013815. ATP_grasp_subdomain_1.
IPR013816. ATP_grasp_subdomain_2.
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.
IPR011054. Rudment_hybrid_motif.
IPR011053. Single_hybrid_motif.
[Graphical view]
PfamiPF02785. Biotin_carb_C. 1 hit.
PF00289. Biotin_carb_N. 1 hit.
PF00364. Biotin_lipoyl. 1 hit.
PF02786. CPSase_L_D2. 1 hit.
[Graphical view]
SMARTiSM00878. Biotin_carb_C. 1 hit.
[Graphical view]
SUPFAMiSSF51230. SSF51230. 1 hit.
SSF51246. SSF51246. 1 hit.
SSF52440. SSF52440. 1 hit.
PROSITEiPS50975. 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.
[Graphical view]

Sequences (3)i

Sequence statusi: Complete.

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

This entry describes 3 isoformsi produced by alternative splicing. AlignAdd to basket

Isoform 1 (identifier: P05165-1) [UniParc]FASTAAdd to basket

This isoform has been chosen as the 'canonical' sequence. All positional information in this entry refers to it. This is also the sequence that appears in the downloadable versions of the entry.

« Hide

        10         20         30         40         50
MAGFWVGTAP LVAAGRRGRW PPQQLMLSAA LRTLKHVLYY SRQCLMVSRN
60 70 80 90 100
LGSVGYDPNE KTFDKILVAN RGEIACRVIR TCKKMGIKTV AIHSDVDASS
110 120 130 140 150
VHVKMADEAV CVGPAPTSKS YLNMDAIMEA IKKTRAQAVH PGYGFLSENK
160 170 180 190 200
EFARCLAAED VVFIGPDTHA IQAMGDKIES KLLAKKAEVN TIPGFDGVVK
210 220 230 240 250
DAEEAVRIAR EIGYPVMIKA SAGGGGKGMR IAWDDEETRD GFRLSSQEAA
260 270 280 290 300
SSFGDDRLLI EKFIDNPRHI EIQVLGDKHG NALWLNEREC SIQRRNQKVV
310 320 330 340 350
EEAPSIFLDA ETRRAMGEQA VALARAVKYS SAGTVEFLVD SKKNFYFLEM
360 370 380 390 400
NTRLQVEHPV TECITGLDLV QEMIRVAKGY PLRHKQADIR INGWAVECRV
410 420 430 440 450
YAEDPYKSFG LPSIGRLSQY QEPLHLPGVR VDSGIQPGSD ISIYYDPMIS
460 470 480 490 500
KLITYGSDRT EALKRMADAL DNYVIRGVTH NIALLREVII NSRFVKGDIS
510 520 530 540 550
TKFLSDVYPD GFKGHMLTKS EKNQLLAIAS SLFVAFQLRA QHFQENSRMP
560 570 580 590 600
VIKPDIANWE LSVKLHDKVH TVVASNNGSV FSVEVDGSKL NVTSTWNLAS
610 620 630 640 650
PLLSVSVDGT QRTVQCLSRE AGGNMSIQFL GTVYKVNILT RLAAELNKFM
660 670 680 690 700
LEKVTEDTSS VLRSPMPGVV VAVSVKPGDA VAEGQEICVI EAMKMQNSMT
710 720
AGKTGTVKSV HCQAGDTVGE GDLLVELE
Length:728
Mass (Da):80,059
Last modified:October 5, 2010 - v4
Checksum:i065F64186A0B8CCC
GO
Isoform 2 (identifier: P05165-2) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     36-61: Missing.

Show »
Length:702
Mass (Da):77,048
Checksum:iB75C63581796ED53
GO
Isoform 3 (identifier: P05165-3) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     634-680: Missing.

Show »
Length:681
Mass (Da):75,002
Checksum:iCF5795BE08D8BD5B
GO

Sequence cautioni

The sequence AAA60035 differs from that shown. Reason: Frameshift at positions 374, 378, 380 and 395. Curated
The sequence AAH00140 differs from that shown. Reason: Erroneous initiation. Translation N-terminally extended.Curated
The sequence AAK61392 differs from that shown. Reason: Erroneous initiation. Translation N-terminally extended.Curated
The sequence CAA32763 differs from that shown. Reason: Frameshift at position 23. Curated

Experimental Info

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Sequence conflicti61 – 611K → E in BAG60571 (PubMed:14702039).Curated
Sequence conflicti93 – 931H → Y in BAG59350 (Ref. 4) Curated
Sequence conflicti373 – 3731M → R in AAA60035 (PubMed:3460076).Curated
Sequence conflicti378 – 3792KG → RS in AAA60035 (PubMed:3460076).Curated
Sequence conflicti558 – 5581N → H in AAA60035 (PubMed:3460076).Curated
Sequence conflicti610 – 6101T → A in BAG59350 (Ref. 4) Curated
Sequence conflicti679 – 6791D → A in AAA36424 (PubMed:3555348).Curated

Natural variant

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Natural varianti75 – 751A → P in PA-1. 1 Publication
Corresponds to variant rs794727479 [ dbSNP | Ensembl ].
VAR_009087
Natural varianti77 – 771R → W in PA-1. 2 Publications
Corresponds to variant rs141371306 [ dbSNP | Ensembl ].
VAR_009088
Natural varianti138 – 1381A → T in PA-1. 1 Publication
Corresponds to variant rs202247814 [ dbSNP | Ensembl ].
VAR_009089
Natural varianti164 – 1641I → T in PA-1. 1 Publication
Corresponds to variant rs202247815 [ dbSNP | Ensembl ].
VAR_009090
Natural varianti197 – 1971G → E in PA-1. 1 Publication
VAR_023843
Natural varianti229 – 2291M → K in PA-1. 1 Publication
VAR_009091
Natural varianti297 – 2971Q → R in PA-1. 1 Publication
VAR_009092
Natural varianti368 – 3681D → G in PA-1. 1 Publication
VAR_009093
Natural varianti373 – 3731M → K in PA-1; unstable protein. 1 Publication
Corresponds to variant rs121964958 [ dbSNP | Ensembl ].
VAR_009094
Natural varianti379 – 3791G → V in PA-1. 1 Publication
Corresponds to variant rs794727087 [ dbSNP | Ensembl ].
VAR_009095
Natural varianti398 – 3981C → R in PA-1. 1 Publication
VAR_023844
Natural varianti399 – 3991R → Q in PA-1. 1 Publication
VAR_009096
Natural varianti423 – 4231P → L in PA-1. 1 Publication
VAR_009097
Natural varianti475 – 4751I → V.
Corresponds to variant rs35719359 [ dbSNP | Ensembl ].
VAR_009098
Natural varianti532 – 5321Missing in PA-1. 1 Publication
VAR_023845
Natural varianti551 – 5511V → F.1 Publication
Corresponds to variant rs61749895 [ dbSNP | Ensembl ].
VAR_023846
Natural varianti559 – 5591W → L in PA-1. 1 Publication
Corresponds to variant rs118169528 [ dbSNP | Ensembl ].
VAR_009099
Natural varianti631 – 6311G → R in PA-1. 1 Publication
Corresponds to variant rs796052018 [ dbSNP | Ensembl ].
VAR_009100
Natural varianti668 – 6681G → R in PA-1. 1 Publication
Corresponds to variant rs771438170 [ dbSNP | Ensembl ].
VAR_009101
Natural varianti712 – 7121Missing in PA-1. 1 Publication
VAR_009102

Alternative sequence

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Alternative sequencei36 – 6126Missing in isoform 2. 1 PublicationVSP_039857Add
BLAST
Alternative sequencei634 – 68047Missing in isoform 3. 1 PublicationVSP_044458Add
BLAST

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AF385926 mRNA. Translation: AAL66189.1.
AY035808
, AY035786, AY035787, AY035788, AY035789, AY035790, AY035791, AY035792, AY035793, AY035794, AY035795, AY035796, AY035797, AY035798, AY035799, AY035800, AY035801, AY035802, AY035803, AY035804, AY035805, AY035806, AY035807 Genomic DNA. Translation: AAK61392.1. Different initiation.
AK296771 mRNA. Translation: BAG59350.1.
AK298318 mRNA. Translation: BAG60571.1.
AL355338
, AL136526, AL353697, AL356575 Genomic DNA. Translation: CAH70370.2.
AL356575
, AL136526, AL353697, AL355338 Genomic DNA. Translation: CAH72681.2.
AL136526
, AL353697, AL355338, AL356575 Genomic DNA. Translation: CAI39557.2.
AL353697
, AL136526, AL355338, AL356575 Genomic DNA. Translation: CAI40434.2.
CH471085 Genomic DNA. Translation: EAX09034.1.
BC000140 mRNA. Translation: AAH00140.1. Different initiation.
X14608 mRNA. Translation: CAA32763.1. Frameshift.
S55656 mRNA. Translation: AAB25345.1.
M13572 mRNA. Translation: AAA60035.1. Frameshift.
M26121 mRNA. Translation: AAA36424.1.
CCDSiCCDS45065.1. [P05165-2]
CCDS53878.1. [P05165-3]
CCDS9496.2. [P05165-1]
PIRiS04613. A27883.
RefSeqiNP_000273.2. NM_000282.3. [P05165-1]
NP_001121164.1. NM_001127692.2. [P05165-2]
NP_001171475.1. NM_001178004.1. [P05165-3]
UniGeneiHs.80741.

Genome annotation databases

EnsembliENST00000376279; ENSP00000365456; ENSG00000175198. [P05165-3]
ENST00000376285; ENSP00000365462; ENSG00000175198. [P05165-1]
ENST00000376286; ENSP00000365463; ENSG00000175198. [P05165-2]
GeneIDi5095.
KEGGihsa:5095.
UCSCiuc001voo.4. human. [P05165-1]

Keywords - Coding sequence diversityi

Alternative splicing, Polymorphism

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AF385926 mRNA. Translation: AAL66189.1.
AY035808
, AY035786, AY035787, AY035788, AY035789, AY035790, AY035791, AY035792, AY035793, AY035794, AY035795, AY035796, AY035797, AY035798, AY035799, AY035800, AY035801, AY035802, AY035803, AY035804, AY035805, AY035806, AY035807 Genomic DNA. Translation: AAK61392.1. Different initiation.
AK296771 mRNA. Translation: BAG59350.1.
AK298318 mRNA. Translation: BAG60571.1.
AL355338
, AL136526, AL353697, AL356575 Genomic DNA. Translation: CAH70370.2.
AL356575
, AL136526, AL353697, AL355338 Genomic DNA. Translation: CAH72681.2.
AL136526
, AL353697, AL355338, AL356575 Genomic DNA. Translation: CAI39557.2.
AL353697
, AL136526, AL355338, AL356575 Genomic DNA. Translation: CAI40434.2.
CH471085 Genomic DNA. Translation: EAX09034.1.
BC000140 mRNA. Translation: AAH00140.1. Different initiation.
X14608 mRNA. Translation: CAA32763.1. Frameshift.
S55656 mRNA. Translation: AAB25345.1.
M13572 mRNA. Translation: AAA60035.1. Frameshift.
M26121 mRNA. Translation: AAA36424.1.
CCDSiCCDS45065.1. [P05165-2]
CCDS53878.1. [P05165-3]
CCDS9496.2. [P05165-1]
PIRiS04613. A27883.
RefSeqiNP_000273.2. NM_000282.3. [P05165-1]
NP_001121164.1. NM_001127692.2. [P05165-2]
NP_001171475.1. NM_001178004.1. [P05165-3]
UniGeneiHs.80741.

3D structure databases

Select the link destinations:
PDBei
RCSB PDBi
PDBji
Links Updated
EntryMethodResolution (Å)ChainPositionsPDBsum
2CQYNMR-A176-270[»]
2JKUX-ray1.50A658-728[»]
ProteinModelPortaliP05165.
SMRiP05165. Positions 63-728.
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi111128. 21 interactions.
DIPiDIP-57493N.
IntActiP05165. 14 interactions.
STRINGi9606.ENSP00000365462.

Chemistry

DrugBankiDB00121. Biotin.

PTM databases

iPTMnetiP05165.
PhosphoSiteiP05165.
SwissPalmiP05165.

Polymorphism and mutation databases

BioMutaiPCCA.
DMDMi308153661.

Proteomic databases

EPDiP05165.
MaxQBiP05165.
PaxDbiP05165.
PeptideAtlasiP05165.
PRIDEiP05165.

Protocols and materials databases

Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENST00000376279; ENSP00000365456; ENSG00000175198. [P05165-3]
ENST00000376285; ENSP00000365462; ENSG00000175198. [P05165-1]
ENST00000376286; ENSP00000365463; ENSG00000175198. [P05165-2]
GeneIDi5095.
KEGGihsa:5095.
UCSCiuc001voo.4. human. [P05165-1]

Organism-specific databases

CTDi5095.
GeneCardsiPCCA.
GeneReviewsiPCCA.
HGNCiHGNC:8653. PCCA.
HPAiHPA041716.
HPA047792.
MalaCardsiPCCA.
MIMi232000. gene.
606054. phenotype.
neXtProtiNX_P05165.
Orphaneti35. Propionic acidemia.
PharmGKBiPA32992.
GenAtlasiSearch...

Phylogenomic databases

eggNOGiKOG0238. Eukaryota.
COG4770. LUCA.
GeneTreeiENSGT00550000074675.
HOGENOMiHOG000008989.
HOVERGENiHBG000555.
InParanoidiP05165.
KOiK01965.
OMAiDIKLKGW.
OrthoDBiEOG091G06RG.
PhylomeDBiP05165.
TreeFamiTF354220.

Enzyme and pathway databases

UniPathwayiUPA00945; UER00908.
BioCyciMetaCyc:ENSG00000175198-MONOMER.
RETL1328306-WGS:GSTH-2235-MONOMER.
ReactomeiR-HSA-196780. Biotin transport and metabolism.
R-HSA-3371599. Defective HLCS causes multiple carboxylase deficiency.
R-HSA-71032. Propionyl-CoA catabolism.
SABIO-RKP05165.

Miscellaneous databases

ChiTaRSiPCCA. human.
EvolutionaryTraceiP05165.
GenomeRNAii5095.
PROiP05165.
SOURCEiSearch...

Gene expression databases

BgeeiENSG00000175198.
CleanExiHS_PCCA.
ExpressionAtlasiP05165. baseline and differential.
GenevisibleiP05165. HS.

Family and domain databases

Gene3Di3.30.1490.20. 1 hit.
3.30.470.20. 1 hit.
3.40.50.20. 1 hit.
InterProiIPR011761. ATP-grasp.
IPR013815. ATP_grasp_subdomain_1.
IPR013816. ATP_grasp_subdomain_2.
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.
IPR011054. Rudment_hybrid_motif.
IPR011053. Single_hybrid_motif.
[Graphical view]
PfamiPF02785. Biotin_carb_C. 1 hit.
PF00289. Biotin_carb_N. 1 hit.
PF00364. Biotin_lipoyl. 1 hit.
PF02786. CPSase_L_D2. 1 hit.
[Graphical view]
SMARTiSM00878. Biotin_carb_C. 1 hit.
[Graphical view]
SUPFAMiSSF51230. SSF51230. 1 hit.
SSF51246. SSF51246. 1 hit.
SSF52440. SSF52440. 1 hit.
PROSITEiPS50975. 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.
[Graphical view]
ProtoNetiSearch...

Entry informationi

Entry nameiPCCA_HUMAN
AccessioniPrimary (citable) accession number: P05165
Secondary accession number(s): B4DKY8
, B4DPF9, C9JPQ8, Q15979, Q8WXQ7
Entry historyi
Integrated into UniProtKB/Swiss-Prot: August 13, 1987
Last sequence update: October 5, 2010
Last modified: September 7, 2016
This is version 193 of the entry and version 4 of the sequence. [Complete history]
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program
DisclaimerAny medical or genetic information present in this entry is provided for research, educational and informational purposes only. It is not in any way intended to be used as a substitute for professional medical advice, diagnosis, treatment or care.

Miscellaneousi

Keywords - Technical termi

3D-structure, Complete proteome, Direct protein sequencing, Reference proteome

Documents

  1. Human chromosome 13
    Human chromosome 13: entries, gene names and cross-references to MIM
  2. Human entries with polymorphisms or disease mutations
    List of human entries with polymorphisms or disease mutations
  3. Human polymorphisms and disease mutations
    Index of human polymorphisms and disease mutations
  4. MIM cross-references
    Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
  5. PATHWAY comments
    Index of metabolic and biosynthesis pathways
  6. PDB cross-references
    Index of Protein Data Bank (PDB) cross-references
  7. SIMILARITY comments
    Index of protein domains and families

Similar proteinsi

Links to similar proteins from the UniProt Reference Clusters (UniRef) at 100%, 90% and 50% sequence identity:
100%UniRef100 combines identical sequences and sub-fragments with 11 or more residues from any organism into one UniRef entry.
90%UniRef90 is built by clustering UniRef100 sequences that have at least 90% sequence identity to, and 80% overlap with, the longest sequence (a.k.a seed sequence).
50%UniRef50 is built by clustering UniRef90 seed sequences that have at least 50% sequence identity to, and 80% overlap with, the longest sequence in the cluster.