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UniProtKB - P05166 (PCCB_HUMAN)

Entry version 190 (13 Feb 2019)
Sequence version 3 (23 Jan 2007)
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Protein

Propionyl-CoA carboxylase beta chain, mitochondrial

Gene

PCCB

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score:

Annotation score:5 out of 5

<p>The annotation score provides a heuristic measure of the annotation content of a UniProtKB entry or proteome. This score <strong>cannot</strong> be used as a measure of the accuracy of the annotation as we cannot define the ‘correct annotation’ for any given protein.<p><a href='/help/annotation_score' target='_top'>More...</a></p>-Experimental evidence at protein leveli <p>This indicates the type of evidence that supports the existence of the protein. Note that the ‘protein existence’ evidence does not give information on the accuracy or correctness of the sequence(s) displayed.<p><a href='/help/protein_existence' target='_top'>More...</a></p>

<p>This section provides any useful information about the protein, mostly biological knowledge.<p><a href='/help/function_section' target='_top'>More...</a></p>Functioni

<p>This subsection of the <a href="http://www.uniprot.org/help/function_section">Function</a> section describes the catalytic activity of an enzyme, i.e. a chemical reaction that the enzyme catalyzes.<p><a href='/help/catalytic_activity' target='_top'>More...</a></p>Catalytic activityi

<p>This subsection of the <a href="http://www.uniprot.org/help/function_section">'Function'</a> section describes the metabolic pathway(s) associated with a protein.<p><a href='/help/pathway' target='_top'>More...</a></p>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.

<p>The <a href="http://www.geneontology.org/">Gene Ontology (GO)</a> project provides a set of hierarchical controlled vocabulary split into 3 categories:<p><a href='/help/gene_ontology' target='_top'>More...</a></p>GO - Molecular functioni

  • ATP binding Source: UniProtKB-KW
  • propionyl-CoA carboxylase activity Source: ProtInc
View the complete GO annotation on QuickGO ...

GO - Biological processi

View the complete GO annotation on QuickGO ...

<p>UniProtKB Keywords constitute a <a href="http://www.uniprot.org/keywords">controlled vocabulary</a> with a hierarchical structure. Keywords summarise the content of a UniProtKB entry and facilitate the search for proteins of interest.<p><a href='/help/keywords' target='_top'>More...</a></p>Keywordsi

Molecular functionLigase
LigandATP-binding, Nucleotide-binding

Enzyme and pathway databases

BioCyc Collection of Pathway/Genome Databases

More...BioCyci		MetaCyc:ENSG00000114054-MONOMER

Reactome - a knowledgebase of biological pathways and processes

More...Reactomei		R-HSA-196780 Biotin transport and metabolism
R-HSA-3371599 Defective HLCS causes multiple carboxylase deficiency
R-HSA-71032 Propionyl-CoA catabolism

SABIO-RK: Biochemical Reaction Kinetics Database

More...SABIO-RKi		P05166

UniPathway: a resource for the exploration and annotation of metabolic pathways

More...UniPathwayi
UPA00945;UER00908

<p>This section provides information about the protein and gene name(s) and synonym(s) and about the organism that is the source of the protein sequence.<p><a href='/help/names_and_taxonomy_section' target='_top'>More...</a></p>Names & Taxonomyi

<p>This subsection of the <a href="http://www.uniprot.org/help/names_and_taxonomy_section">Names and taxonomy</a> section provides an exhaustive list of all names of the protein, from commonly used to obsolete, to allow unambiguous identification of a protein.<p><a href='/help/protein_names' target='_top'>More...</a></p>Protein namesi
Recommended name:
Propionyl-CoA carboxylase beta chain, mitochondrial (EC:6.4.1.3)
Short name:
PCCase subunit beta
Alternative name(s):
Propanoyl-CoA:carbon dioxide ligase subunit beta
<p>This subsection of the <a href="http://www.uniprot.org/help/names_and_taxonomy_section">Names and taxonomy</a> section indicates the name(s) of the gene(s) that code for the protein sequence(s) described in the entry. Four distinct tokens exist: ‘Name’, ‘Synonyms’, ‘Ordered locus names’ and ‘ORF names’.<p><a href='/help/gene_name' target='_top'>More...</a></p>Gene namesi
Name:PCCB
<p>This subsection of the <a href="http://www.uniprot.org/help/names_and_taxonomy_section">Names and taxonomy</a> section provides information on the name(s) of the organism that is the source of the protein sequence.<p><a href='/help/organism-name' target='_top'>More...</a></p>OrganismiHomo sapiens (Human)
<p>This subsection of the <a href="http://www.uniprot.org/help/names_and_taxonomy_section">Names and taxonomy</a> section shows the unique identifier assigned by the NCBI to the source organism of the protein. This is known as the ‘taxonomic identifier’ or ‘taxid’.<p><a href='/help/taxonomic_identifier' target='_top'>More...</a></p>Taxonomic identifieri9606 [NCBI]
<p>This subsection of the <a href="http://www.uniprot.org/help/names_and_taxonomy_section">Names and taxonomy</a> section contains the taxonomic hierarchical classification lineage of the source organism. It lists the nodes as they appear top-down in the taxonomic tree, with the more general grouping listed first.<p><a href='/help/taxonomic_lineage' target='_top'>More...</a></p>Taxonomic lineageiEukaryota › Metazoa › Chordata › Craniata › Vertebrata › Euteleostomi › Mammalia › Eutheria › Euarchontoglires › Primates › Haplorrhini › Catarrhini › Hominidae › Homo
<p>This subsection of the <a href="http://www.uniprot.org/help/names_and_taxonomy_section">Names and taxonomy</a> section is present for entries that are part of a <a href="http://www.uniprot.org/proteomes">proteome</a>, i.e. of a set of proteins thought to be expressed by organisms whose genomes have been completely sequenced.<p><a href='/help/proteomes_manual' target='_top'>More...</a></p>Proteomesi
  • UP000005640 <p>A UniProt <a href="http://www.uniprot.org/manual/proteomes_manual">proteome</a> can consist of several components. <br></br>The component name refers to the genomic component encoding a set of proteins.<p><a href='/help/proteome_component' target='_top'>More...</a></p> Componenti: Chromosome 3

Organism-specific databases

Eukaryotic Pathogen Database Resources

More...EuPathDBi		HostDB:ENSG00000114054.13

Human Gene Nomenclature Database

More...HGNCi		HGNC:8654 PCCB

Online Mendelian Inheritance in Man (OMIM)

More...MIMi		232050 gene

neXtProt; the human protein knowledge platform

More...neXtProti		NX_P05166

<p>This section provides information on the location and the topology of the mature protein in the cell.<p><a href='/help/subcellular_location_section' target='_top'>More...</a></p>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 & Seán O’Donoghue; Source: COMPARTMENTS

Keywords - Cellular componenti

Mitochondrion

<p>This section provides information on the disease(s) and phenotype(s) associated with a protein.<p><a href='/help/pathology_and_biotech_section' target='_top'>More...</a></p>Pathology & Biotechi

<p>This subsection of the ‘Pathology and Biotech’ section provides information on the disease(s) associated with genetic variations in a given protein. The information is extracted from the scientific literature and diseases that are also described in the <a href="http://www.ncbi.nlm.nih.gov/sites/entrez?db=omim">OMIM</a> database are represented with a <a href="http://www.uniprot.org/diseases">controlled vocabulary</a> in the following way:<p><a href='/help/involvement_in_disease' target='_top'>More...</a></p>Involvement in diseasei

Propionic acidemia type II (PA-2)8 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)DescriptionActionsGraphical viewLength
<p>This subsection of the ‘Sequence’ section describes natural variant(s) of the protein sequence.<p><a href='/help/variant' target='_top'>More...</a></p>Natural variantiVAR_00908017L → M in PA-2. 1 PublicationCorresponds to variant dbSNP:rs200185747EnsemblClinVar.1
Natural variantiVAR_00027144R → P in PA-2. 1
Natural variantiVAR_02384767R → S in PA-2. 1 PublicationCorresponds to variant dbSNP:rs747053913Ensembl.1
Natural variantiVAR_000272106S → R in PA-2. 1
Natural variantiVAR_023848107V → M in PA-2. 1 Publication1
Natural variantiVAR_023849112G → D in PA-2. 1 PublicationCorresponds to variant dbSNP:rs202247818EnsemblClinVar.1
Natural variantiVAR_000273131G → R in PA-2. 1
Natural variantiVAR_009081140K → KICK in PA-2. 1
Natural variantiVAR_023850153A → P in PA-2. 1 PublicationCorresponds to variant dbSNP:rs202247819EnsemblClinVar.1
Natural variantiVAR_023851165R → Q in PA-2; does not affect either heteromeric or homomeric assembly. 2 PublicationsCorresponds to variant dbSNP:rs1304714042Ensembl.1
Natural variantiVAR_000274165R → W in PA-2. 1 PublicationCorresponds to variant dbSNP:rs879253815EnsemblClinVar.1
Natural variantiVAR_000275168E → K in PA-2; common mutation. 2 PublicationsCorresponds to variant dbSNP:rs121964960EnsemblClinVar.1
Natural variantiVAR_023852188G → R in PA-2. 1 PublicationCorresponds to variant dbSNP:rs746102997EnsemblClinVar.1
Natural variantiVAR_000276198G → D in PA-2. Corresponds to variant dbSNP:rs762354873Ensembl.1
Natural variantiVAR_009082205V → D in PA-2. 1 Publication1
Natural variantiVAR_009083228P → L in PA-2. Corresponds to variant dbSNP:rs374722096EnsemblClinVar.1
Natural variantiVAR_023853246G → V in PA-2. 1 Publication1
Natural variantiVAR_023854341Missing in PA-2. 1 Publication1
Natural variantiVAR_000277408Missing in PA-2. 2 Publications1
Natural variantiVAR_000278410R → W in PA-2. 3 PublicationsCorresponds to variant dbSNP:rs121964959EnsemblClinVar.1
Natural variantiVAR_009084428T → I in PA-2. 2 PublicationsCorresponds to variant dbSNP:rs111033542EnsemblClinVar.1
Natural variantiVAR_023855430I → L in PA-2. 1 Publication1
Natural variantiVAR_023856435Y → C in PA-2. 1 PublicationCorresponds to variant dbSNP:rs121964961EnsemblClinVar.1
Natural variantiVAR_023857439Y → C in PA-2. 1 PublicationCorresponds to variant dbSNP:rs769521436EnsemblClinVar.1
Natural variantiVAR_009085442M → T in PA-2. 1 Publication1
Natural variantiVAR_023858468A → T in PA-2. 1 PublicationCorresponds to variant dbSNP:rs775563122EnsemblClinVar.1
Natural variantiVAR_000279497A → V in PA-2; common mutation; does not affect either heteromeric or homomeric assembly. 1 PublicationCorresponds to variant dbSNP:rs142403318EnsemblClinVar.1
Natural variantiVAR_000280512R → C in PA-2; affects heteromeric and homomeric assembly. 2 PublicationsCorresponds to variant dbSNP:rs186710233EnsemblClinVar.1
Natural variantiVAR_000281519L → P in PA-2; affects heteromeric and homomeric assembly. 1 PublicationCorresponds to variant dbSNP:rs202247822EnsemblClinVar.1
Natural variantiVAR_009086536N → D in PA-2; affects heteromeric and homomeric assembly. 1 PublicationCorresponds to variant dbSNP:rs202247823EnsemblClinVar.1

Keywords - Diseasei

Disease mutation

Organism-specific databases

DisGeNET

More...DisGeNETi		5096

GeneReviews a resource of expert-authored, peer-reviewed disease descriptions.

More...GeneReviewsi		PCCB

MalaCards human disease database

More...MalaCardsi		PCCB
MIMi606054 phenotype

Open Targets

More...OpenTargetsi		ENSG00000114054

Orphanet; a database dedicated to information on rare diseases and orphan drugs

More...Orphaneti		35 Propionic acidemia

The Pharmacogenetics and Pharmacogenomics Knowledge Base

More...PharmGKBi		PA32993

Chemistry databases

Drug and drug target database

More...DrugBanki		DB00121 Biotin
DB00161 L-Valine

Polymorphism and mutation databases

BioMuta curated single-nucleotide variation and disease association database

More...BioMutai		PCCB

Domain mapping of disease mutations (DMDM)

More...DMDMi		124106304

<p>This section describes post-translational modifications (PTMs) and/or processing events.<p><a href='/help/ptm_processing_section' target='_top'>More...</a></p>PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the ‘PTM / Processing’ section describes the extent of a transit peptide.<p><a href='/help/transit' target='_top'>More...</a></p>Transit peptidei1 – 28Mitochondrion1 PublicationAdd BLAST28
<p>This subsection of the ‘PTM / Processing’ section describes the extent of a polypeptide chain in the mature protein following processing.<p><a href='/help/chain' target='_top'>More...</a></p>ChainiPRO_000000029329 – 539Propionyl-CoA carboxylase beta chain, mitochondrialAdd BLAST511

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the ‘PTM / Processing’ section specifies the position and type of each modified residue excluding <a href="http://www.uniprot.org/manual/lipid">lipids</a>, <a href="http://www.uniprot.org/manual/carbohyd">glycans</a> and <a href="http://www.uniprot.org/manual/crosslnk">protein cross-links</a>.<p><a href='/help/mod_res' target='_top'>More...</a></p>Modified residuei71PhosphoserineCombined sources1
Modified residuei99N6-acetyllysine; alternateBy similarity1
Modified residuei99N6-succinyllysine; alternateBy similarity1
Modified residuei248N6-succinyllysineBy similarity1
Modified residuei474N6-acetyllysine; alternateBy similarity1
Modified residuei474N6-succinyllysine; alternateBy similarity1
Modified residuei489N6-acetyllysine; alternateBy similarity1
Modified residuei489N6-succinyllysine; alternateBy similarity1

Keywords - PTMi

Acetylation, Phosphoprotein

Proteomic databases

Encyclopedia of Proteome Dynamics

More...EPDi		P05166

jPOST - Japan Proteome Standard Repository/Database

More...jPOSTi		P05166

MaxQB - The MaxQuant DataBase

More...MaxQBi		P05166

PaxDb, a database of protein abundance averages across all three domains of life

More...PaxDbi		P05166

PeptideAtlas

More...PeptideAtlasi		P05166

PRoteomics IDEntifications database

More...PRIDEi		P05166

ProteomicsDB human proteome resource

More...ProteomicsDBi		51816
51817 [P05166-2]

PTM databases

iPTMnet integrated resource for PTMs in systems biology context

More...iPTMneti		P05166

Comprehensive resource for the study of protein post-translational modifications (PTMs) in human, mouse and rat.

More...PhosphoSitePlusi		P05166

<p>This section provides information on the expression of a gene at the mRNA or protein level in cells or in tissues of multicellular organisms.<p><a href='/help/expression_section' target='_top'>More...</a></p>Expressioni

Gene expression databases

Bgee dataBase for Gene Expression Evolution

More...Bgeei		ENSG00000114054 Expressed in 222 organ(s), highest expression level in right adrenal gland

ExpressionAtlas, Differential and Baseline Expression

More...ExpressionAtlasi		P05166 baseline and differential

Genevisible search portal to normalized and curated expression data from Genevestigator

More...Genevisiblei		P05166 HS

Organism-specific databases

Human Protein Atlas

More...HPAi		HPA036939
HPA036940

<p>This section provides information on the quaternary structure of a protein and on interaction(s) with other proteins or protein complexes.<p><a href='/help/interaction_section' target='_top'>More...</a></p>Interactioni

<p>This subsection of the <a href="http://www.uniprot.org/help/interaction_section">'Interaction'</a> section provides information about the protein quaternary structure and interaction(s) with other proteins or protein complexes (with the exception of physiological receptor-ligand interactions which are annotated in the <a href="http://www.uniprot.org/help/function_section">'Function'</a> section).<p><a href='/help/subunit_structure' target='_top'>More...</a></p>Subunit structurei

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

<p>This subsection of the '<a href="http://www.uniprot.org/help/interaction_section%27">Interaction</a> section provides information about binary protein-protein interactions. The data presented in this section are a quality-filtered subset of binary interactions automatically derived from the <a href="http://www.ebi.ac.uk/intact/">IntAct database</a>. It is updated on a monthly basis. Each binary interaction is displayed on a separate line.<p><a href='/help/binary_interactions' target='_top'>More...</a></p>Binary interactionsi

WithEntry#Exp.IntActNotes
PCCAP051653EBI-1371908,EBI-2211679

Protein-protein interaction databases

The Biological General Repository for Interaction Datasets (BioGrid)

More...BioGridi		111129, 23 interactors

CORUM comprehensive resource of mammalian protein complexes

More...CORUMi		P05166

Database of interacting proteins

More...DIPi		DIP-38244N

Protein interaction database and analysis system

More...IntActi		P05166, 12 interactors

Molecular INTeraction database

More...MINTi		P05166

STRING: functional protein association networks

More...STRINGi		9606.ENSP00000419027

<p>This section provides information on the tertiary and secondary structure of a protein.<p><a href='/help/structure_section' target='_top'>More...</a></p>Structurei

3D structure databases

Protein Model Portal of the PSI-Nature Structural Biology Knowledgebase

More...ProteinModelPortali		P05166

SWISS-MODEL Repository - a database of annotated 3D protein structure models

More...SMRi		P05166

Database of comparative protein structure models

More...ModBasei		Search...

MobiDB: a database of protein disorder and mobility annotations

More...MobiDBi		Search...

<p>This section provides information on sequence similarities with other proteins and the domain(s) present in a protein.<p><a href='/help/family_and_domains_section' target='_top'>More...</a></p>Family & Domainsi

Domains and Repeats

Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the <a href="http://www.uniprot.org/help/family_and_domains_section">Family and Domains</a> section describes the position and type of a domain, which is defined as a specific combination of secondary structures organized into a characteristic three-dimensional structure or fold.<p><a href='/help/domain' target='_top'>More...</a></p>Domaini32 – 290CoA carboxyltransferase N-terminalPROSITE-ProRule annotationAdd BLAST259
Domaini294 – 533CoA carboxyltransferase C-terminalPROSITE-ProRule annotationAdd BLAST240

Region

Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the ‘Family and Domains’ section describes a region of interest that cannot be described in other subsections.<p><a href='/help/region' target='_top'>More...</a></p>Regioni32 – 533CarboxyltransferasePROSITE-ProRule annotationAdd BLAST502
Regioni325 – 358Acyl-CoA bindingSequence analysisAdd BLAST34

<p>This subsection of the ‘Family and domains’ section provides information about the sequence similarity with other proteins.<p><a href='/help/sequence_similarities' target='_top'>More...</a></p>Sequence similaritiesi

Belongs to the AccD/PCCB family.Curated

Keywords - Domaini

Transit peptide

Phylogenomic databases

evolutionary genealogy of genes: Non-supervised Orthologous Groups

More...eggNOGi		KOG0540 Eukaryota
COG4799 LUCA

Ensembl GeneTree

More...GeneTreei		ENSGT00940000157741

The HOGENOM Database of Homologous Genes from Fully Sequenced Organisms

More...HOGENOMi		HOG000218693

The HOVERGEN Database of Homologous Vertebrate Genes

More...HOVERGENi		HBG003970

InParanoid: Eukaryotic Ortholog Groups

More...InParanoidi		P05166

KEGG Orthology (KO)

More...KOi		K01966

Database of Orthologous Groups

More...OrthoDBi		589058at2759

Database for complete collections of gene phylogenies

More...PhylomeDBi		P05166

TreeFam database of animal gene trees

More...TreeFami		TF314350

Family and domain databases

Integrated resource of protein families, domains and functional sites

More...InterProi		View protein in InterPro
IPR034733 AcCoA_carboxyl
IPR029045 ClpP/crotonase-like_dom_sf
IPR011763 COA_CT_C
IPR011762 COA_CT_N

Pfam protein domain database

More...Pfami		View protein in Pfam
PF01039 Carboxyl_trans, 1 hit

Superfamily database of structural and functional annotation

More...SUPFAMi		SSF52096 SSF52096, 2 hits

PROSITE; a protein domain and family database

More...PROSITEi		View protein in PROSITE
PS50989 COA_CT_CTER, 1 hit
PS50980 COA_CT_NTER, 1 hit

<p>This section displays by default the canonical protein sequence and upon request all isoforms described in the entry. It also includes information pertinent to the sequence(s), including <a href="http://www.uniprot.org/help/sequence_length">length</a> and <a href="http://www.uniprot.org/help/sequences">molecular weight</a>.<p><a href='/help/sequences_section' target='_top'>More...</a></p>Sequences (2+)i

<p>This subsection of the <a href="http://www.uniprot.org/help/sequences_section">Sequence</a> section indicates if the <a href="http://www.uniprot.org/help/canonical_and_isoforms">canonical sequence</a> displayed by default in the entry is complete or not.<p><a href='/help/sequence_status' target='_top'>More...</a></p>Sequence statusi: Complete.

<p>This subsection of the <a href="http://www.uniprot.org/help/sequences_section">Sequence</a> section indicates if the <a href="http://www.uniprot.org/help/canonical_and_isoforms">canonical sequence</a> displayed by default in the entry is in its mature form or if it represents the precursor.<p><a href='/help/sequence_processing' target='_top'>More...</a></p>Sequence processingi: The displayed sequence is further processed into a mature form.

This entry describes 2 <p>This subsection of the ‘Sequence’ section lists the alternative protein sequences (isoforms) that can be generated from the same gene by a single or by the combination of up to four biological events (alternative promoter usage, alternative splicing, alternative initiation and ribosomal frameshifting). Additionally, this section gives relevant information on each alternative protein isoform.<p><a href='/help/alternative_products' target='_top'>More...</a></p> isoformsi produced by alternative splicing. AlignAdd to basket

This entry has 2 described isoforms and 13 potential isoforms that are computationally mapped.Show allAlign All

Isoform 1 (identifier: P05166-1) [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
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
Length:539
Mass (Da):58,216
Last modified:January 23, 2007 - v3
<p>The checksum is a form of redundancy check that is calculated from the sequence. It is useful for tracking sequence updates.</p> <p>It should be noted that while, in theory, two different sequences could have the same checksum value, the likelihood that this would happen is extremely low.</p> <p>However UniProtKB may contain entries with identical sequences in case of multiple genes (paralogs).</p> <p>The checksum is computed as the sequence 64-bit Cyclic Redundancy Check value (CRC64) using the generator polynomial: x<sup>64</sup> + x<sup>4</sup> + x<sup>3</sup> + x + 1. The algorithm is described in the ISO 3309 standard. </p> <p class="publication">Press W.H., Flannery B.P., Teukolsky S.A. and Vetterling W.T.<br /> <strong>Cyclic redundancy and other checksums</strong><br /> <a href="http://www.nrbook.com/b/bookcpdf.php">Numerical recipes in C 2nd ed., pp896-902, Cambridge University Press (1993)</a>)</p> Checksum:iA2DAAC00312D3C0F
GO
Isoform 2 (identifier: P05166-2) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     124-124: Q → QQIIGWAQWLPLVISALWEAE

Note: No experimental confirmation available.
Show »
Length:559
Mass (Da):60,521
Checksum:i07788B2E955626AA
GO

<p>In eukaryotic reference proteomes, unreviewed entries that are likely to belong to the same gene are computationally mapped, based on gene identifiers from Ensembl, EnsemblGenomes and model organism databases.<p><a href='/help/gene_centric_isoform_mapping' target='_top'>More...</a></p>Computationally mapped potential isoform sequencesi

There are 13 potential isoforms mapped to this entry.BLASTAlignShow allAdd to basket
EntryEntry nameProtein names
Gene namesLengthAnnotation
E7EX59E7EX59_HUMAN
Propionyl-CoA carboxylase beta chai...
PCCB
570Annotation score:

Annotation score:1 out of 5

<p>The annotation score provides a heuristic measure of the annotation content of a UniProtKB entry or proteome. This score <strong>cannot</strong> be used as a measure of the accuracy of the annotation as we cannot define the ‘correct annotation’ for any given protein.<p><a href='/help/annotation_score' target='_top'>More...</a></p>
E7ETT4E7ETT4_HUMAN
Propionyl-CoA carboxylase beta chai...
PCCB
482Annotation score:

Annotation score:1 out of 5

<p>The annotation score provides a heuristic measure of the annotation content of a UniProtKB entry or proteome. This score <strong>cannot</strong> be used as a measure of the accuracy of the annotation as we cannot define the ‘correct annotation’ for any given protein.<p><a href='/help/annotation_score' target='_top'>More...</a></p>
E9PDR0E9PDR0_HUMAN
Propionyl-CoA carboxylase beta chai...
PCCB
550Annotation score:

Annotation score:1 out of 5

<p>The annotation score provides a heuristic measure of the annotation content of a UniProtKB entry or proteome. This score <strong>cannot</strong> be used as a measure of the accuracy of the annotation as we cannot define the ‘correct annotation’ for any given protein.<p><a href='/help/annotation_score' target='_top'>More...</a></p>
C9JQS9C9JQS9_HUMAN
Propionyl-CoA carboxylase beta chai...
PCCB
559Annotation score:

Annotation score:1 out of 5

<p>The annotation score provides a heuristic measure of the annotation content of a UniProtKB entry or proteome. This score <strong>cannot</strong> be used as a measure of the accuracy of the annotation as we cannot define the ‘correct annotation’ for any given protein.<p><a href='/help/annotation_score' target='_top'>More...</a></p>
F8WBI9F8WBI9_HUMAN
Propionyl-CoA carboxylase beta chai...
PCCB
405Annotation score:

Annotation score:1 out of 5

<p>The annotation score provides a heuristic measure of the annotation content of a UniProtKB entry or proteome. This score <strong>cannot</strong> be used as a measure of the accuracy of the annotation as we cannot define the ‘correct annotation’ for any given protein.<p><a href='/help/annotation_score' target='_top'>More...</a></p>
E9PEC3E9PEC3_HUMAN
Propionyl-CoA carboxylase beta chai...
PCCB
423Annotation score:

Annotation score:1 out of 5

<p>The annotation score provides a heuristic measure of the annotation content of a UniProtKB entry or proteome. This score <strong>cannot</strong> be used as a measure of the accuracy of the annotation as we cannot define the ‘correct annotation’ for any given protein.<p><a href='/help/annotation_score' target='_top'>More...</a></p>
C9JVW9C9JVW9_HUMAN
Propionyl-CoA carboxylase beta chai...
PCCB
133Annotation score:

Annotation score:1 out of 5

<p>The annotation score provides a heuristic measure of the annotation content of a UniProtKB entry or proteome. This score <strong>cannot</strong> be used as a measure of the accuracy of the annotation as we cannot define the ‘correct annotation’ for any given protein.<p><a href='/help/annotation_score' target='_top'>More...</a></p>
E7EUY3E7EUY3_HUMAN
Propionyl-CoA carboxylase beta chai...
PCCB
516Annotation score:

Annotation score:1 out of 5

<p>The annotation score provides a heuristic measure of the annotation content of a UniProtKB entry or proteome. This score <strong>cannot</strong> be used as a measure of the accuracy of the annotation as we cannot define the ‘correct annotation’ for any given protein.<p><a href='/help/annotation_score' target='_top'>More...</a></p>
H7C5C9H7C5C9_HUMAN
Propionyl-CoA carboxylase beta chai...
PCCB
163Annotation score:

Annotation score:1 out of 5

<p>The annotation score provides a heuristic measure of the annotation content of a UniProtKB entry or proteome. This score <strong>cannot</strong> be used as a measure of the accuracy of the annotation as we cannot define the ‘correct annotation’ for any given protein.<p><a href='/help/annotation_score' target='_top'>More...</a></p>
E7ETT1E7ETT1_HUMAN
Propionyl-CoA carboxylase beta chai...
PCCB
520Annotation score:

Annotation score:1 out of 5

<p>The annotation score provides a heuristic measure of the annotation content of a UniProtKB entry or proteome. This score <strong>cannot</strong> be used as a measure of the accuracy of the annotation as we cannot define the ‘correct annotation’ for any given protein.<p><a href='/help/annotation_score' target='_top'>More...</a></p>
There are more potential isoformsShow all

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the ‘Sequence’ section reports difference(s) between the canonical sequence (displayed by default in the entry) and the different sequence submissions merged in the entry. These various submissions may originate from different sequencing projects, different types of experiments, or different biological samples. Sequence conflicts are usually of unknown origin.<p><a href='/help/conflict' target='_top'>More...</a></p>Sequence conflicti58 – 59QH → HD in AAB28900 (PubMed:8225321).Curated2

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_00908017L → M in PA-2. 1 PublicationCorresponds to variant dbSNP:rs200185747EnsemblClinVar.1
Natural variantiVAR_00027144R → P in PA-2. 1
Natural variantiVAR_02384767R → S in PA-2. 1 PublicationCorresponds to variant dbSNP:rs747053913Ensembl.1
Natural variantiVAR_000272106S → R in PA-2. 1
Natural variantiVAR_023848107V → M in PA-2. 1 Publication1
Natural variantiVAR_023849112G → D in PA-2. 1 PublicationCorresponds to variant dbSNP:rs202247818EnsemblClinVar.1
Natural variantiVAR_000273131G → R in PA-2. 1
Natural variantiVAR_009081140K → KICK in PA-2. 1
Natural variantiVAR_023850153A → P in PA-2. 1 PublicationCorresponds to variant dbSNP:rs202247819EnsemblClinVar.1
Natural variantiVAR_023851165R → Q in PA-2; does not affect either heteromeric or homomeric assembly. 2 PublicationsCorresponds to variant dbSNP:rs1304714042Ensembl.1
Natural variantiVAR_000274165R → W in PA-2. 1 PublicationCorresponds to variant dbSNP:rs879253815EnsemblClinVar.1
Natural variantiVAR_000275168E → K in PA-2; common mutation. 2 PublicationsCorresponds to variant dbSNP:rs121964960EnsemblClinVar.1
Natural variantiVAR_023852188G → R in PA-2. 1 PublicationCorresponds to variant dbSNP:rs746102997EnsemblClinVar.1
Natural variantiVAR_000276198G → D in PA-2. Corresponds to variant dbSNP:rs762354873Ensembl.1
Natural variantiVAR_009082205V → D in PA-2. 1 Publication1
Natural variantiVAR_009083228P → L in PA-2. Corresponds to variant dbSNP:rs374722096EnsemblClinVar.1
Natural variantiVAR_023853246G → V in PA-2. 1 Publication1
Natural variantiVAR_048163287P → S1 PublicationCorresponds to variant dbSNP:rs2228310Ensembl.1
Natural variantiVAR_023854341Missing in PA-2. 1 Publication1
Natural variantiVAR_000277408Missing in PA-2. 2 Publications1
Natural variantiVAR_000278410R → W in PA-2. 3 PublicationsCorresponds to variant dbSNP:rs121964959EnsemblClinVar.1
Natural variantiVAR_009084428T → I in PA-2. 2 PublicationsCorresponds to variant dbSNP:rs111033542EnsemblClinVar.1
Natural variantiVAR_023855430I → L in PA-2. 1 Publication1
Natural variantiVAR_023856435Y → C in PA-2. 1 PublicationCorresponds to variant dbSNP:rs121964961EnsemblClinVar.1
Natural variantiVAR_023857439Y → C in PA-2. 1 PublicationCorresponds to variant dbSNP:rs769521436EnsemblClinVar.1
Natural variantiVAR_009085442M → T in PA-2. 1 Publication1
Natural variantiVAR_023858468A → T in PA-2. 1 PublicationCorresponds to variant dbSNP:rs775563122EnsemblClinVar.1
Natural variantiVAR_000279497A → V in PA-2; common mutation; does not affect either heteromeric or homomeric assembly. 1 PublicationCorresponds to variant dbSNP:rs142403318EnsemblClinVar.1
Natural variantiVAR_000280512R → C in PA-2; affects heteromeric and homomeric assembly. 2 PublicationsCorresponds to variant dbSNP:rs186710233EnsemblClinVar.1
Natural variantiVAR_000281519L → P in PA-2; affects heteromeric and homomeric assembly. 1 PublicationCorresponds to variant dbSNP:rs202247822EnsemblClinVar.1
Natural variantiVAR_009086536N → D in PA-2; affects heteromeric and homomeric assembly. 1 PublicationCorresponds to variant dbSNP:rs202247823EnsemblClinVar.1

Alternative sequence

Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the ‘Sequence’ section describes the sequence of naturally occurring alternative protein isoform(s). The changes in the amino acid sequence may be due to alternative splicing, alternative promoter usage, alternative initiation, or ribosomal frameshifting.<p><a href='/help/var_seq' target='_top'>More...</a></p>Alternative sequenceiVSP_042568124Q → QQIIGWAQWLPLVISALWEA E in isoform 2. 1 Publication1

Sequence databases

Select the link destinations:

EMBL nucleotide sequence database

More...EMBLi

GenBank nucleotide sequence database

More...GenBanki

DNA Data Bank of Japan; a nucleotide sequence database

More...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

The Consensus CDS (CCDS) project

More...CCDSi		CCDS3089.1 [P05166-1]
CCDS54643.1 [P05166-2]

Protein sequence database of the Protein Information Resource

More...PIRi		T45009

NCBI Reference Sequences

More...RefSeqi		NP_000523.2, NM_000532.4 [P05166-1]
NP_001171485.1, NM_001178014.1 [P05166-2]

UniGene gene-oriented nucleotide sequence clusters

More...UniGenei		Hs.63788

Genome annotation databases

Ensembl eukaryotic genome annotation project

More...Ensembli		ENST00000251654; ENSP00000251654; ENSG00000114054 [P05166-1]
ENST00000469217; ENSP00000419027; ENSG00000114054 [P05166-2]

Database of genes from NCBI RefSeq genomes

More...GeneIDi		5096

KEGG: Kyoto Encyclopedia of Genes and Genomes

More...KEGGi		hsa:5096

UCSC genome browser

More...UCSCi		uc003eqy.3 human [P05166-1]

Keywords - Coding sequence diversityi

Alternative splicing, Polymorphism

<p>This section provides links to proteins that are similar to the protein sequence(s) described in this entry at different levels of sequence identity thresholds (100%, 90% and 50%) based on their membership in UniProt Reference Clusters (<a href="http://www.uniprot.org/help/uniref">UniRef</a>).<p><a href='/help/similar_proteins_section' target='_top'>More...</a></p>Similar proteinsi

<p>This section is used to point to information related to entries and found in data collections other than UniProtKB.<p><a href='/help/cross_references_section' target='_top'>More...</a></p>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
CCDSiCCDS3089.1 [P05166-1]
CCDS54643.1 [P05166-2]
PIRiT45009
RefSeqiNP_000523.2, NM_000532.4 [P05166-1]
NP_001171485.1, NM_001178014.1 [P05166-2]
UniGeneiHs.63788

3D structure databases

ProteinModelPortaliP05166
SMRiP05166
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi111129, 23 interactors
CORUMiP05166
DIPiDIP-38244N
IntActiP05166, 12 interactors
MINTiP05166
STRINGi9606.ENSP00000419027

Chemistry databases

DrugBankiDB00121 Biotin
DB00161 L-Valine

PTM databases

iPTMnetiP05166
PhosphoSitePlusiP05166

Polymorphism and mutation databases

BioMutaiPCCB
DMDMi124106304

Proteomic databases

EPDiP05166
jPOSTiP05166
MaxQBiP05166
PaxDbiP05166
PeptideAtlasiP05166
PRIDEiP05166
ProteomicsDBi51816
51817 [P05166-2]

Protocols and materials databases

The DNASU plasmid repository

More...DNASUi		5096
Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENST00000251654; ENSP00000251654; ENSG00000114054 [P05166-1]
ENST00000469217; ENSP00000419027; ENSG00000114054 [P05166-2]
GeneIDi5096
KEGGihsa:5096
UCSCiuc003eqy.3 human [P05166-1]

Organism-specific databases

Comparative Toxicogenomics Database

More...CTDi		5096
DisGeNETi5096
EuPathDBiHostDB:ENSG00000114054.13

GeneCards: human genes, protein and diseases

More...GeneCardsi		PCCB
GeneReviewsiPCCB
HGNCiHGNC:8654 PCCB
HPAiHPA036939
HPA036940
MalaCardsiPCCB
MIMi232050 gene
606054 phenotype
neXtProtiNX_P05166
OpenTargetsiENSG00000114054
Orphaneti35 Propionic acidemia
PharmGKBiPA32993

GenAtlas: human gene database

More...GenAtlasi		Search...

Phylogenomic databases

eggNOGiKOG0540 Eukaryota
COG4799 LUCA
GeneTreeiENSGT00940000157741
HOGENOMiHOG000218693
HOVERGENiHBG003970
InParanoidiP05166
KOiK01966
OrthoDBi589058at2759
PhylomeDBiP05166
TreeFamiTF314350

Enzyme and pathway databases

UniPathwayi
UPA00945;UER00908

BioCyciMetaCyc:ENSG00000114054-MONOMER
ReactomeiR-HSA-196780 Biotin transport and metabolism
R-HSA-3371599 Defective HLCS causes multiple carboxylase deficiency
R-HSA-71032 Propionyl-CoA catabolism
SABIO-RKiP05166

Miscellaneous databases

ChiTaRS: a database of human, mouse and fruit fly chimeric transcripts and RNA-sequencing data

More...ChiTaRSi		PCCB human

Database of phenotypes from RNA interference screens in Drosophila and Homo sapiens

More...GenomeRNAii		5096

Protein Ontology

More...PROi		PR:P05166

The Stanford Online Universal Resource for Clones and ESTs

More...SOURCEi		Search...

Gene expression databases

BgeeiENSG00000114054 Expressed in 222 organ(s), highest expression level in right adrenal gland
ExpressionAtlasiP05166 baseline and differential
GenevisibleiP05166 HS

Family and domain databases

InterProiView protein in InterPro
IPR034733 AcCoA_carboxyl
IPR029045 ClpP/crotonase-like_dom_sf
IPR011763 COA_CT_C
IPR011762 COA_CT_N
PfamiView protein in Pfam
PF01039 Carboxyl_trans, 1 hit
SUPFAMiSSF52096 SSF52096, 2 hits
PROSITEiView protein in PROSITE
PS50989 COA_CT_CTER, 1 hit
PS50980 COA_CT_NTER, 1 hit

ProtoNet; Automatic hierarchical classification of proteins

More...ProtoNeti		Search...

<p>This section provides general information on the entry.<p><a href='/help/entry_information_section' target='_top'>More...</a></p>Entry informationi

<p>This subsection of the ‘Entry information’ section provides a mnemonic identifier for a UniProtKB entry, but it is not a stable identifier. Each reviewed entry is assigned a unique entry name upon integration into UniProtKB/Swiss-Prot.<p><a href='/help/entry_name' target='_top'>More...</a></p>Entry nameiPCCB_HUMAN
<p>This subsection of the ‘Entry information’ section provides one or more accession number(s). These are stable identifiers and should be used to cite UniProtKB entries. Upon integration into UniProtKB, each entry is assigned a unique accession number, which is called ‘Primary (citable) accession number’.<p><a href='/help/accession_numbers' target='_top'>More...</a></p>AccessioniPrimary (citable) accession number: P05166
Secondary accession number(s): B7Z2Z4, Q16813, Q96CX0
<p>This subsection of the ‘Entry information’ section shows the date of integration of the entry into UniProtKB, the date of the last sequence update and the date of the last annotation modification (‘Last modified’). The version number for both the entry and the <a href="http://www.uniprot.org/help/canonical_and_isoforms">canonical sequence</a> are also displayed.<p><a href='/help/entry_history' target='_top'>More...</a></p>Entry historyiIntegrated into UniProtKB/Swiss-Prot: August 13, 1987
Last sequence update: January 23, 2007
Last modified: February 13, 2019
This is version 190 of the entry and version 3 of the sequence. See complete history.
<p>This subsection of the ‘Entry information’ section indicates whether the entry has been manually annotated and reviewed by UniProtKB curators or not, in other words, if the entry belongs to the Swiss-Prot section of UniProtKB (<strong>reviewed</strong>) or to the computer-annotated TrEMBL section (<strong>unreviewed</strong>).<p><a href='/help/entry_status' target='_top'>More...</a></p>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.

<p>This section contains any relevant information that doesn’t fit in any other defined sections<p><a href='/help/miscellaneous_section' target='_top'>More...</a></p>Miscellaneousi

Keywords - Technical termi

Complete proteome, Direct protein sequencing, Reference proteome

Documents

  1. SIMILARITY comments
    Index of protein domains and families
  2. Human chromosome 3
    Human chromosome 3: entries, gene names and cross-references to MIM
  3. Human entries with polymorphisms or disease mutations
    List of human entries with polymorphisms or disease mutations
  4. Human polymorphisms and disease mutations
    Index of human polymorphisms and disease mutations
  5. MIM cross-references
    Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
  6. PATHWAY comments
    Index of metabolic and biosynthesis pathways
UniProt is an ELIXIR core data resource
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