UniProtKB - P05166 (PCCB_HUMAN)
Propionyl-CoA carboxylase beta chain, mitochondrial
PCCB
Functioni
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 (PubMed:6765947, PubMed:15890657).
Propionyl-CoA carboxylase catalyzes the carboxylation of propionyl-CoA/propanoyl-CoA to D-methylmalonyl-CoA/(S)-methylmalonyl-CoA (PubMed:6765947, PubMed:15890657).
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 (By similarity).
Propionyl-CoA carboxylase also significantly acts on butyryl-CoA/butanoyl-CoA, which is converted to ethylmalonyl-CoA/(2S)-ethylmalonyl-CoA at a much lower rate (PubMed:6765947).
Other alternative minor substrates include (2E)-butenoyl-CoA/crotonoyl-CoA (By similarity).
By similarity2 PublicationsCatalytic activityi
- ATP + hydrogencarbonate + propanoyl-CoA = (S)-methylmalonyl-CoA + ADP + H+ + phosphate2 PublicationsEC:6.4.1.32 PublicationsThis reaction proceeds in the forward2 Publications direction.
- This reaction proceeds in the forwardBy similarity direction.
Kineticsi
- KM=0.29 mM for propanoyl-CoA1 Publication
- KM=0.41 mM for propanoyl-CoA (at 37 degrees Celsius and pH 8.0)1 Publication
pH dependencei
: propanoyl-CoA degradation Pathwayi
This protein is involved in step 1 of the subpathway that synthesizes succinyl-CoA from propanoyl-CoA.2 Publications 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.
GO - Molecular functioni
- ATP binding Source: UniProtKB-KW
- propionyl-CoA carboxylase activity Source: UniProtKB
GO - Biological processi
- branched-chain amino acid metabolic process Source: ComplexPortal
- fatty acid metabolic process Source: ComplexPortal
- short-chain fatty acid catabolic process Source: UniProtKB
Keywordsi
Molecular function | Ligase |
Ligand | ATP-binding, Nucleotide-binding |
Enzyme and pathway databases
BRENDAi | 6.4.1.3, 2681 |
PathwayCommonsi | P05166 |
Reactomei | R-HSA-196780, Biotin transport and metabolism R-HSA-3371599, Defective HLCS causes multiple carboxylase deficiency R-HSA-71032, Propionyl-CoA catabolism |
SABIO-RKi | P05166 |
SignaLinki | P05166 |
SIGNORi | P05166 |
UniPathwayi | UPA00945;UER00908 |
Names & Taxonomyi
Protein namesi | Recommended name: Propionyl-CoA carboxylase beta chain, mitochondrial1 Publication (EC:6.4.1.32 Publications)Short name: PCCase subunit beta Alternative name(s): Propanoyl-CoA:carbon dioxide ligase subunit beta |
Gene namesi | Name:PCCBImported |
Organismi | Homo sapiens (Human) |
Taxonomic identifieri | 9606 [NCBI] |
Taxonomic lineagei | Eukaryota › Metazoa › Chordata › Craniata › Vertebrata › Euteleostomi › Mammalia › Eutheria › Euarchontoglires › Primates › Haplorrhini › Catarrhini › Hominidae › Homo |
Proteomesi |
|
Organism-specific databases
HGNCi | HGNC:8654, PCCB |
MIMi | 232050, gene |
neXtProti | NX_P05166 |
VEuPathDBi | HostDB:ENSG00000114054 |
Subcellular locationi
Mitochondrion
- Mitochondrion matrix 1 Publication
Cytosol
- cytosol Source: Reactome
Mitochondrion
- mitochondrial matrix Source: UniProtKB
- mitochondrion Source: GO_Central
Other locations
- catalytic complex Source: ComplexPortal
Keywords - Cellular componenti
MitochondrionPathology & Biotechi
Involvement in diseasei
Propionic acidemia type II (PA-2)9 Publications
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Natural variantiVAR_009080 | 17 | L → M in PA-2; likely benign variant. 1 PublicationCorresponds to variant dbSNP:rs200185747EnsemblClinVar. | 1 | |
Natural variantiVAR_000271 | 44 | R → P in PA-2. | 1 | |
Natural variantiVAR_023847 | 67 | R → S in PA-2. 1 PublicationCorresponds to variant dbSNP:rs747053913Ensembl. | 1 | |
Natural variantiVAR_000272 | 106 | S → R in PA-2. | 1 | |
Natural variantiVAR_023848 | 107 | V → M in PA-2. 1 PublicationCorresponds to variant dbSNP:rs1553774114EnsemblClinVar. | 1 | |
Natural variantiVAR_023849 | 112 | G → D in PA-2. 1 PublicationCorresponds to variant dbSNP:rs202247818EnsemblClinVar. | 1 | |
Natural variantiVAR_000273 | 131 | G → R in PA-2. | 1 | |
Natural variantiVAR_009081 | 140 | K → KICK in PA-2. | 1 | |
Natural variantiVAR_023850 | 153 | A → P in PA-2. 1 PublicationCorresponds to variant dbSNP:rs202247819EnsemblClinVar. | 1 | |
Natural variantiVAR_023851 | 165 | R → Q in PA-2; does not affect either heteromeric or homomeric assembly. 2 PublicationsCorresponds to variant dbSNP:rs1304714042EnsemblClinVar. | 1 | |
Natural variantiVAR_000274 | 165 | R → W in PA-2; no effect on affinity for propionyl-CoA; decreased reaction kinetics for the propionyl-CoA carboxylase activity; decreased thermostability affecting holoenzyme oligomerization. 2 PublicationsCorresponds to variant dbSNP:rs879253815EnsemblClinVar. | 1 | |
Natural variantiVAR_000275 | 168 | E → K in PA-2; no effect on affinity for propionyl-CoA; decreased reaction kinetics for the propionyl-CoA carboxylase activity; decreased thermostability affecting holoenzyme oligomerization. 3 PublicationsCorresponds to variant dbSNP:rs121964960EnsemblClinVar. | 1 | |
Natural variantiVAR_023852 | 188 | G → R in PA-2. 1 PublicationCorresponds to variant dbSNP:rs746102997EnsemblClinVar. | 1 | |
Natural variantiVAR_000276 | 198 | G → D in PA-2. Corresponds to variant dbSNP:rs762354873Ensembl. | 1 | |
Natural variantiVAR_009082 | 205 | V → D in PA-2. 1 Publication | 1 | |
Natural variantiVAR_009083 | 228 | P → L in PA-2. Corresponds to variant dbSNP:rs374722096EnsemblClinVar. | 1 | |
Natural variantiVAR_023853 | 246 | G → V in PA-2. 1 Publication | 1 | |
Natural variantiVAR_023854 | 341 | Missing in PA-2. 1 Publication | 1 | |
Natural variantiVAR_000277 | 408 | Missing in PA-2. 2 Publications | 1 | |
Natural variantiVAR_000278 | 410 | R → W in PA-2; no effect on affinity for propionyl-CoA; decreased reaction kinetics for the propionyl-CoA carboxylase activity; decreased thermostability affecting holoenzyme oligomerization. 4 PublicationsCorresponds to variant dbSNP:rs121964959EnsemblClinVar. | 1 | |
Natural variantiVAR_009084 | 428 | T → I in PA-2. 2 PublicationsCorresponds to variant dbSNP:rs111033542EnsemblClinVar. | 1 | |
Natural variantiVAR_023855 | 430 | I → L in PA-2. 1 Publication | 1 | |
Natural variantiVAR_023856 | 435 | Y → C in PA-2. 1 PublicationCorresponds to variant dbSNP:rs121964961EnsemblClinVar. | 1 | |
Natural variantiVAR_023857 | 439 | Y → C in PA-2. 1 PublicationCorresponds to variant dbSNP:rs769521436EnsemblClinVar. | 1 | |
Natural variantiVAR_009085 | 442 | M → T in PA-2. 1 Publication | 1 | |
Natural variantiVAR_023858 | 468 | A → T in PA-2. 1 PublicationCorresponds to variant dbSNP:rs775563122EnsemblClinVar. | 1 | |
Natural variantiVAR_000280 | 512 | R → C in PA-2; affects heteromeric and homomeric assembly. 2 PublicationsCorresponds to variant dbSNP:rs186710233EnsemblClinVar. | 1 | |
Natural variantiVAR_000281 | 519 | L → P in PA-2; affects heteromeric and homomeric assembly. 1 PublicationCorresponds to variant dbSNP:rs202247822EnsemblClinVar. | 1 | |
Natural variantiVAR_009086 | 536 | N → D in PA-2; affects heteromeric and homomeric assembly. 1 PublicationCorresponds to variant dbSNP:rs202247823EnsemblClinVar. | 1 |
Keywords - Diseasei
Disease variantOrganism-specific databases
DisGeNETi | 5096 |
GeneReviewsi | PCCB |
MalaCardsi | PCCB |
MIMi | 606054, phenotype |
OpenTargetsi | ENSG00000114054 |
Orphaneti | 35, Propionic acidemia |
PharmGKBi | PA32993 |
Miscellaneous databases
Pharosi | P05166, Tbio |
Chemistry databases
DrugBanki | DB00121, Biotin DB00161, Valine |
Genetic variation databases
BioMutai | PCCB |
DMDMi | 124106304 |
PTM / Processingi
Molecule processing
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Transit peptidei | 1 – 28 | Mitochondrion1 PublicationAdd BLAST | 28 | |
ChainiPRO_0000000293 | 29 – 539 | Propionyl-CoA carboxylase beta chain, mitochondrialAdd BLAST | 511 |
Amino acid modifications
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Modified residuei | 71 | PhosphoserineCombined sources | 1 | |
Modified residuei | 99 | N6-acetyllysine; alternateBy similarity | 1 | |
Modified residuei | 99 | N6-succinyllysine; alternateBy similarity | 1 | |
Modified residuei | 248 | N6-succinyllysineBy similarity | 1 | |
Modified residuei | 474 | N6-acetyllysine; alternateBy similarity | 1 | |
Modified residuei | 474 | N6-succinyllysine; alternateBy similarity | 1 | |
Modified residuei | 489 | N6-acetyllysine; alternateBy similarity | 1 | |
Modified residuei | 489 | N6-succinyllysine; alternateBy similarity | 1 |
Keywords - PTMi
Acetylation, PhosphoproteinProteomic databases
EPDi | P05166 |
jPOSTi | P05166 |
MassIVEi | P05166 |
MaxQBi | P05166 |
PaxDbi | P05166 |
PeptideAtlasi | P05166 |
PRIDEi | P05166 |
ProteomicsDBi | 51816 [P05166-1] 51817 [P05166-2] |
PTM databases
iPTMneti | P05166 |
PhosphoSitePlusi | P05166 |
Expressioni
Gene expression databases
Bgeei | ENSG00000114054, Expressed in right adrenal gland and 239 other tissues |
ExpressionAtlasi | P05166, baseline and differential |
Genevisiblei | P05166, HS |
Organism-specific databases
HPAi | ENSG00000114054, Tissue enhanced (liver) |
Interactioni
Subunit structurei
The holoenzyme is a dodecamer composed of 6 PCCA/alpha subunits and 6 PCCB/beta subunits.
2 PublicationsBinary interactionsi
Protein-protein interaction databases
BioGRIDi | 111129, 136 interactors |
ComplexPortali | CPX-6169, Mitochondrial propionyl-CoA carboxylase complex |
CORUMi | P05166 |
DIPi | DIP-38244N |
IntActi | P05166, 25 interactors |
MINTi | P05166 |
STRINGi | 9606.ENSP00000419027 |
Miscellaneous databases
RNActi | P05166, protein |
Family & Domainsi
Domains and Repeats
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Domaini | 32 – 290 | CoA carboxyltransferase N-terminalPROSITE-ProRule annotationAdd BLAST | 259 | |
Domaini | 294 – 533 | CoA carboxyltransferase C-terminalPROSITE-ProRule annotationAdd BLAST | 240 |
Region
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Regioni | 32 – 533 | CarboxyltransferasePROSITE-ProRule annotationAdd BLAST | 502 | |
Regioni | 325 – 358 | Acyl-CoA bindingSequence analysisAdd BLAST | 34 |
Domaini
Sequence similaritiesi
Keywords - Domaini
Transit peptidePhylogenomic databases
eggNOGi | KOG0540, Eukaryota |
GeneTreei | ENSGT00940000157741 |
InParanoidi | P05166 |
OMAi | YAYAECT |
OrthoDBi | 402617at2759 |
PhylomeDBi | P05166 |
TreeFami | TF314350 |
Family and domain databases
InterProi | View protein in InterPro IPR034733, AcCoA_carboxyl IPR029045, ClpP/crotonase-like_dom_sf IPR011763, COA_CT_C IPR011762, COA_CT_N |
Pfami | View protein in Pfam PF01039, Carboxyl_trans, 1 hit |
SUPFAMi | SSF52096, SSF52096, 2 hits |
PROSITEi | View protein in PROSITE PS50989, COA_CT_CTER, 1 hit PS50980, COA_CT_NTER, 1 hit |
s (2+)i Sequence
Sequence statusi: Complete.
: The displayed sequence is further processed into a mature form. Sequence processingi
This entry describes 2 produced by isoformsialternative splicing. AlignAdd to basketThis entry has 2 described isoforms and 13 potential isoforms that are computationally mapped.Show allAlign All
This isoform has been chosen as the sequence. All positional information in this entry refers to it. This is also the sequence that appears in the downloadable versions of the entry. canonicali
10 20 30 40 50
MAAALRVAAV GARLSVLASG LRAAVRSLCS QATSVNERIE NKRRTALLGG
60 70 80 90 100
GQRRIDAQHK RGKLTARERI SLLLDPGSFV ESDMFVEHRC ADFGMAADKN
110 120 130 140 150
KFPGDSVVTG RGRINGRLVY VFSQDFTVFG GSLSGAHAQK ICKIMDQAIT
160 170 180 190 200
VGAPVIGLND SGGARIQEGV ESLAGYADIF LRNVTASGVI PQISLIMGPC
210 220 230 240 250
AGGAVYSPAL TDFTFMVKDT SYLFITGPDV VKSVTNEDVT QEELGGAKTH
260 270 280 290 300
TTMSGVAHRA FENDVDALCN LRDFFNYLPL SSQDPAPVRE CHDPSDRLVP
310 320 330 340 350
ELDTIVPLES TKAYNMVDII HSVVDEREFF EIMPNYAKNI IVGFARMNGR
360 370 380 390 400
TVGIVGNQPK VASGCLDINS SVKGARFVRF CDAFNIPLIT FVDVPGFLPG
410 420 430 440 450
TAQEYGGIIR HGAKLLYAFA EATVPKVTVI TRKAYGGAYD VMSSKHLCGD
460 470 480 490 500
TNYAWPTAEI AVMGAKGAVE IIFKGHENVE AAQAEYIEKF ANPFPAAVRG
510 520 530
FVDDIIQPSS TRARICCDLD VLASKKVQRP WRKHANIPL
Computationally mapped potential isoform sequencesi
There are 13 potential isoforms mapped to this entry.BLASTAlignShow allAdd to basketC9JVW9 | C9JVW9_HUMAN | Propionyl-CoA carboxylase beta chai... | PCCB | 133 | Annotation score: | ||
H7C5C9 | H7C5C9_HUMAN | Propionyl-CoA carboxylase beta chai... | PCCB | 163 | Annotation score: | ||
E7EX59 | E7EX59_HUMAN | Propionyl-CoA carboxylase beta chai... | PCCB | 570 | Annotation score: | ||
C9JQS9 | C9JQS9_HUMAN | Propionyl-CoA carboxylase beta chai... | PCCB | 559 | Annotation score: | ||
E7EUY3 | E7EUY3_HUMAN | Propionyl-CoA carboxylase beta chai... | PCCB | 516 | Annotation score: | ||
E7ETT1 | E7ETT1_HUMAN | Propionyl-CoA carboxylase beta chai... | PCCB | 520 | Annotation score: | ||
E9PDR0 | E9PDR0_HUMAN | Propionyl-CoA carboxylase beta chai... | PCCB | 550 | Annotation score: | ||
E9PEC3 | E9PEC3_HUMAN | Propionyl-CoA carboxylase beta chai... | PCCB | 423 | Annotation score: | ||
E7ETT4 | E7ETT4_HUMAN | Propionyl-CoA carboxylase beta chai... | PCCB | 482 | Annotation score: | ||
F8WBI9 | F8WBI9_HUMAN | Propionyl-CoA carboxylase beta chai... | PCCB | 405 | Annotation score: | ||
There are more potential isoformsShow all |
Experimental Info
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Sequence conflicti | 58 – 59 | QH → HD in AAB28900 (PubMed:8225321).Curated | 2 |
Natural variant
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Natural variantiVAR_009080 | 17 | L → M in PA-2; likely benign variant. 1 PublicationCorresponds to variant dbSNP:rs200185747EnsemblClinVar. | 1 | |
Natural variantiVAR_000271 | 44 | R → P in PA-2. | 1 | |
Natural variantiVAR_023847 | 67 | R → S in PA-2. 1 PublicationCorresponds to variant dbSNP:rs747053913Ensembl. | 1 | |
Natural variantiVAR_000272 | 106 | S → R in PA-2. | 1 | |
Natural variantiVAR_023848 | 107 | V → M in PA-2. 1 PublicationCorresponds to variant dbSNP:rs1553774114EnsemblClinVar. | 1 | |
Natural variantiVAR_023849 | 112 | G → D in PA-2. 1 PublicationCorresponds to variant dbSNP:rs202247818EnsemblClinVar. | 1 | |
Natural variantiVAR_000273 | 131 | G → R in PA-2. | 1 | |
Natural variantiVAR_009081 | 140 | K → KICK in PA-2. | 1 | |
Natural variantiVAR_023850 | 153 | A → P in PA-2. 1 PublicationCorresponds to variant dbSNP:rs202247819EnsemblClinVar. | 1 | |
Natural variantiVAR_023851 | 165 | R → Q in PA-2; does not affect either heteromeric or homomeric assembly. 2 PublicationsCorresponds to variant dbSNP:rs1304714042EnsemblClinVar. | 1 | |
Natural variantiVAR_000274 | 165 | R → W in PA-2; no effect on affinity for propionyl-CoA; decreased reaction kinetics for the propionyl-CoA carboxylase activity; decreased thermostability affecting holoenzyme oligomerization. 2 PublicationsCorresponds to variant dbSNP:rs879253815EnsemblClinVar. | 1 | |
Natural variantiVAR_000275 | 168 | E → K in PA-2; no effect on affinity for propionyl-CoA; decreased reaction kinetics for the propionyl-CoA carboxylase activity; decreased thermostability affecting holoenzyme oligomerization. 3 PublicationsCorresponds to variant dbSNP:rs121964960EnsemblClinVar. | 1 | |
Natural variantiVAR_023852 | 188 | G → R in PA-2. 1 PublicationCorresponds to variant dbSNP:rs746102997EnsemblClinVar. | 1 | |
Natural variantiVAR_000276 | 198 | G → D in PA-2. Corresponds to variant dbSNP:rs762354873Ensembl. | 1 | |
Natural variantiVAR_009082 | 205 | V → D in PA-2. 1 Publication | 1 | |
Natural variantiVAR_009083 | 228 | P → L in PA-2. Corresponds to variant dbSNP:rs374722096EnsemblClinVar. | 1 | |
Natural variantiVAR_023853 | 246 | G → V in PA-2. 1 Publication | 1 | |
Natural variantiVAR_048163 | 287 | P → S1 PublicationCorresponds to variant dbSNP:rs2228310Ensembl. | 1 | |
Natural variantiVAR_023854 | 341 | Missing in PA-2. 1 Publication | 1 | |
Natural variantiVAR_000277 | 408 | Missing in PA-2. 2 Publications | 1 | |
Natural variantiVAR_000278 | 410 | R → W in PA-2; no effect on affinity for propionyl-CoA; decreased reaction kinetics for the propionyl-CoA carboxylase activity; decreased thermostability affecting holoenzyme oligomerization. 4 PublicationsCorresponds to variant dbSNP:rs121964959EnsemblClinVar. | 1 | |
Natural variantiVAR_009084 | 428 | T → I in PA-2. 2 PublicationsCorresponds to variant dbSNP:rs111033542EnsemblClinVar. | 1 | |
Natural variantiVAR_023855 | 430 | I → L in PA-2. 1 Publication | 1 | |
Natural variantiVAR_023856 | 435 | Y → C in PA-2. 1 PublicationCorresponds to variant dbSNP:rs121964961EnsemblClinVar. | 1 | |
Natural variantiVAR_023857 | 439 | Y → C in PA-2. 1 PublicationCorresponds to variant dbSNP:rs769521436EnsemblClinVar. | 1 | |
Natural variantiVAR_009085 | 442 | M → T in PA-2. 1 Publication | 1 | |
Natural variantiVAR_023858 | 468 | A → T in PA-2. 1 PublicationCorresponds to variant dbSNP:rs775563122EnsemblClinVar. | 1 | |
Natural variantiVAR_000279 | 497 | A → V Found in patients with propionic acidemia; does not affect either heteromeric or homomeric assembly; decreased thermostability affecting holoenzyme oligomerization; no effect on propionyl-CoA carboxylase activity. 1 PublicationCorresponds to variant dbSNP:rs142403318EnsemblClinVar. | 1 | |
Natural variantiVAR_000280 | 512 | R → C in PA-2; affects heteromeric and homomeric assembly. 2 PublicationsCorresponds to variant dbSNP:rs186710233EnsemblClinVar. | 1 | |
Natural variantiVAR_000281 | 519 | L → P in PA-2; affects heteromeric and homomeric assembly. 1 PublicationCorresponds to variant dbSNP:rs202247822EnsemblClinVar. | 1 | |
Natural variantiVAR_009086 | 536 | N → D in PA-2; affects heteromeric and homomeric assembly. 1 PublicationCorresponds to variant dbSNP:rs202247823EnsemblClinVar. | 1 |
Alternative sequence
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Alternative sequenceiVSP_042568 | 124 | Q → QQIIGWAQWLPLVISALWEA E in isoform 2. 1 Publication | 1 |
Sequence databases
Select the link destinations: EMBLi GenBanki DDBJi Links Updated | X73424 mRNA Translation: CAA51825.1 S67325 mRNA Translation: AAB28900.1 AJ006487 , AJ006488, AJ006489, AJ006490, AJ006491, AJ006492, AJ006493, AJ006494, AJ006495, AJ006496, AJ006497, AJ006498, AJ006499 Genomic DNA Translation: CAA07066.1 AK295312 mRNA Translation: BAH12030.1 AC069524 Genomic DNA No translation available. CH471052 Genomic DNA Translation: EAW79118.1 BC013768 mRNA Translation: AAH13768.1 BC053661 mRNA Translation: AAH53661.1 M13573 mRNA Translation: AAA60036.1 M31167 Genomic DNA Translation: AAA60037.1 M31169 Genomic DNA Translation: AAA60038.1 |
CCDSi | CCDS3089.1 [P05166-1] CCDS54643.1 [P05166-2] |
PIRi | T45009 |
RefSeqi | NP_000523.2, NM_000532.4 [P05166-1] NP_001171485.1, NM_001178014.1 [P05166-2] |
Genome annotation databases
Ensembli | ENST00000251654.9; ENSP00000251654.4; ENSG00000114054.14 ENST00000469217.5; ENSP00000419027.1; ENSG00000114054.14 [P05166-2] |
GeneIDi | 5096 |
KEGGi | hsa:5096 |
MANE-Selecti | ENST00000251654.9; ENSP00000251654.4; NM_000532.5; NP_000523.2 |
UCSCi | uc003eqy.3, human [P05166-1] |
Keywords - Coding sequence diversityi
Alternative splicingSimilar proteinsi
Cross-referencesi
Sequence databases
Select the link destinations: EMBLi GenBanki DDBJi Links Updated | X73424 mRNA Translation: CAA51825.1 S67325 mRNA Translation: AAB28900.1 AJ006487 , AJ006488, AJ006489, AJ006490, AJ006491, AJ006492, AJ006493, AJ006494, AJ006495, AJ006496, AJ006497, AJ006498, AJ006499 Genomic DNA Translation: CAA07066.1 AK295312 mRNA Translation: BAH12030.1 AC069524 Genomic DNA No translation available. CH471052 Genomic DNA Translation: EAW79118.1 BC013768 mRNA Translation: AAH13768.1 BC053661 mRNA Translation: AAH53661.1 M13573 mRNA Translation: AAA60036.1 M31167 Genomic DNA Translation: AAA60037.1 M31169 Genomic DNA Translation: AAA60038.1 |
CCDSi | CCDS3089.1 [P05166-1] CCDS54643.1 [P05166-2] |
PIRi | T45009 |
RefSeqi | NP_000523.2, NM_000532.4 [P05166-1] NP_001171485.1, NM_001178014.1 [P05166-2] |
3D structure databases
AlphaFoldDBi | P05166 |
SMRi | P05166 |
ModBasei | Search... |
Protein-protein interaction databases
BioGRIDi | 111129, 136 interactors |
ComplexPortali | CPX-6169, Mitochondrial propionyl-CoA carboxylase complex |
CORUMi | P05166 |
DIPi | DIP-38244N |
IntActi | P05166, 25 interactors |
MINTi | P05166 |
STRINGi | 9606.ENSP00000419027 |
Chemistry databases
DrugBanki | DB00121, Biotin DB00161, Valine |
PTM databases
iPTMneti | P05166 |
PhosphoSitePlusi | P05166 |
Genetic variation databases
BioMutai | PCCB |
DMDMi | 124106304 |
Proteomic databases
EPDi | P05166 |
jPOSTi | P05166 |
MassIVEi | P05166 |
MaxQBi | P05166 |
PaxDbi | P05166 |
PeptideAtlasi | P05166 |
PRIDEi | P05166 |
ProteomicsDBi | 51816 [P05166-1] 51817 [P05166-2] |
Protocols and materials databases
Antibodypediai | 33408, 213 antibodies from 30 providers |
DNASUi | 5096 |
Genome annotation databases
Ensembli | ENST00000251654.9; ENSP00000251654.4; ENSG00000114054.14 ENST00000469217.5; ENSP00000419027.1; ENSG00000114054.14 [P05166-2] |
GeneIDi | 5096 |
KEGGi | hsa:5096 |
MANE-Selecti | ENST00000251654.9; ENSP00000251654.4; NM_000532.5; NP_000523.2 |
UCSCi | uc003eqy.3, human [P05166-1] |
Organism-specific databases
CTDi | 5096 |
DisGeNETi | 5096 |
GeneCardsi | PCCB |
GeneReviewsi | PCCB |
HGNCi | HGNC:8654, PCCB |
HPAi | ENSG00000114054, Tissue enhanced (liver) |
MalaCardsi | PCCB |
MIMi | 232050, gene 606054, phenotype |
neXtProti | NX_P05166 |
OpenTargetsi | ENSG00000114054 |
Orphaneti | 35, Propionic acidemia |
PharmGKBi | PA32993 |
VEuPathDBi | HostDB:ENSG00000114054 |
GenAtlasi | Search... |
Phylogenomic databases
eggNOGi | KOG0540, Eukaryota |
GeneTreei | ENSGT00940000157741 |
InParanoidi | P05166 |
OMAi | YAYAECT |
OrthoDBi | 402617at2759 |
PhylomeDBi | P05166 |
TreeFami | TF314350 |
Enzyme and pathway databases
UniPathwayi | UPA00945;UER00908 |
BRENDAi | 6.4.1.3, 2681 |
PathwayCommonsi | P05166 |
Reactomei | R-HSA-196780, Biotin transport and metabolism R-HSA-3371599, Defective HLCS causes multiple carboxylase deficiency R-HSA-71032, Propionyl-CoA catabolism |
SABIO-RKi | P05166 |
SignaLinki | P05166 |
SIGNORi | P05166 |
Miscellaneous databases
BioGRID-ORCSi | 5096, 11 hits in 1079 CRISPR screens |
ChiTaRSi | PCCB, human |
GenomeRNAii | 5096 |
Pharosi | P05166, Tbio |
PROi | PR:P05166 |
RNActi | P05166, protein |
SOURCEi | Search... |
Gene expression databases
Bgeei | ENSG00000114054, Expressed in right adrenal gland and 239 other tissues |
ExpressionAtlasi | P05166, baseline and differential |
Genevisiblei | P05166, HS |
Family and domain databases
InterProi | View protein in InterPro IPR034733, AcCoA_carboxyl IPR029045, ClpP/crotonase-like_dom_sf IPR011763, COA_CT_C IPR011762, COA_CT_N |
Pfami | View protein in Pfam PF01039, Carboxyl_trans, 1 hit |
SUPFAMi | SSF52096, SSF52096, 2 hits |
PROSITEi | View protein in PROSITE PS50989, COA_CT_CTER, 1 hit PS50980, COA_CT_NTER, 1 hit |
MobiDBi | Search... |
Entry informationi
Entry namei | PCCB_HUMAN | |
Accessioni | P05166Primary (citable) accession number: P05166 Secondary accession number(s): B7Z2Z4, Q16813, Q96CX0 | |
Entry historyi | Integrated into UniProtKB/Swiss-Prot: | August 13, 1987 |
Last sequence update: | January 23, 2007 | |
Last modified: | May 25, 2022 | |
This is version 207 of the entry and version 3 of the sequence. See complete history. | ||
Entry statusi | Reviewed (UniProtKB/Swiss-Prot) | |
Annotation program | Chordata Protein Annotation Program | |
Disclaimer | Any 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
Direct protein sequencing, Reference proteomeDocuments
- Human chromosome 3
Human chromosome 3: entries, gene names and cross-references to MIM - Human entries with genetic variants
List of human entries with genetic variants - Human variants curated from literature reports
Index of human variants curated from literature reports - MIM cross-references
Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot - PATHWAY comments
Index of metabolic and biosynthesis pathways - SIMILARITY comments
Index of protein domains and families