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    PCCA propionyl-CoA carboxylase subunit alpha [ Homo sapiens (human) ]

    Gene ID: 5095, updated on 3-Apr-2024

    Summary

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    Official Symbol
    PCCAprovided by HGNC
    Official Full Name
    propionyl-CoA carboxylase subunit alphaprovided by HGNC
    Primary source
    HGNC:HGNC:8653
    See related
    Ensembl:ENSG00000175198 MIM:232000; AllianceGenome:HGNC:8653
    Gene type
    protein coding
    RefSeq status
    REVIEWED
    Organism
    Homo sapiens
    Lineage
    Eukaryota; Metazoa; Chordata; Craniata; Vertebrata; Euteleostomi; Mammalia; Eutheria; Euarchontoglires; Primates; Haplorrhini; Catarrhini; Hominidae; Homo
    Summary
    The protein encoded by this gene is the alpha subunit of the heterodimeric mitochondrial enzyme Propionyl-CoA carboxylase. PCCA encodes the biotin-binding region of this enzyme. Mutations in either PCCA or PCCB (encoding the beta subunit) lead to an enzyme deficiency resulting in propionic acidemia. Multiple transcript variants encoding different isoforms have been found for this gene.[provided by RefSeq, May 2010]
    Expression
    Broad expression in kidney (RPKM 11.9), liver (RPKM 5.1) and 23 other tissues See more
    Orthologs
    mouse all
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    Genomic context

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    Location:
    13q32.3
    Exon count:
    34
    Annotation release Status Assembly Chr Location
    RS_2023_10 current GRCh38.p14 (GCF_000001405.40) 13 NC_000013.11 (100089093..100530435)
    RS_2023_10 current T2T-CHM13v2.0 (GCF_009914755.1) 13 NC_060937.1 (99302579..99745721)
    105.20220307 previous assembly GRCh37.p13 (GCF_000001405.25) 13 NC_000013.10 (100741347..101182689)

    Chromosome 13 - NC_000013.11Genomic Context describing neighboring genes Neighboring gene OCT4-NANOG hESC enhancer GRCh37_chr13:100732007-100732527 Neighboring gene ATAC-STARR-seq lymphoblastoid active region 7958 Neighboring gene ATAC-STARR-seq lymphoblastoid active region 7959 Neighboring gene ATAC-STARR-seq lymphoblastoid silent region 5476 Neighboring gene Sharpr-MPRA regulatory region 14306 Neighboring gene ASNS pseudogene 3 Neighboring gene PCCA divergent transcript Neighboring gene OCT4-H3K27ac hESC enhancer GRCh37_chr13:100781340-100781840 Neighboring gene OCT4-H3K27ac hESC enhancer GRCh37_chr13:100781841-100782341 Neighboring gene OCT4-NANOG-H3K27ac hESC enhancer GRCh37_chr13:100800509-100801160 Neighboring gene H3K27ac-H3K4me1 hESC enhancer GRCh37_chr13:100803131-100803672 Neighboring gene H3K27ac-H3K4me1 hESC enhancer GRCh37_chr13:100802588-100803130 Neighboring gene H3K4me1 hESC enhancer GRCh37_chr13:100811517-100812018 Neighboring gene H3K4me1 hESC enhancer GRCh37_chr13:100812019-100812518 Neighboring gene ribosomal protein L15 pseudogene 18 Neighboring gene H3K27ac hESC enhancer GRCh37_chr13:100849737-100850237 Neighboring gene H3K27ac hESC enhancer GRCh37_chr13:100875184-100875695 Neighboring gene H3K27ac-H3K4me1 hESC enhancer GRCh37_chr13:100875696-100876206 Neighboring gene uncharacterized LOC105370335 Neighboring gene Sharpr-MPRA regulatory region 7114 Neighboring gene uncharacterized LOC124903201 Neighboring gene P300/CBP strongly-dependent group 1 enhancer GRCh37_chr13:101044561-101045760 Neighboring gene uncharacterized LOC107984574 Neighboring gene H3K27ac hESC enhancer GRCh37_chr13:101145271-101145771 Neighboring gene H3K27ac hESC enhancer GRCh37_chr13:101144770-101145270 Neighboring gene PCCA antisense RNA 1 Neighboring gene H3K27ac-H3K4me1 hESC enhancer GRCh37_chr13:101154747-101155384 Neighboring gene RPL7L1 pseudogene 6 Neighboring gene NANOG-H3K27ac-H3K4me1 hESC enhancer GRCh37_chr13:101161543-101162161 Neighboring gene OCT4-NANOG-H3K27ac hESC enhancer GRCh37_chr13:101162162-101162780 Neighboring gene OCT4-NANOG-H3K27ac-H3K4me1 hESC enhancer GRCh37_chr13:101162781-101163398 Neighboring gene H3K4me1 hESC enhancer GRCh37_chr13:101169316-101170040 Neighboring gene H3K4me1 hESC enhancer GRCh37_chr13:101171491-101172156 Neighboring gene H3K4me1 hESC enhancer GRCh37_chr13:101177366-101177888 Neighboring gene H3K4me1 hESC enhancer GRCh37_chr13:101182623-101183592 Neighboring gene ATAC-STARR-seq lymphoblastoid active region 7960 Neighboring gene H3K4me1 hESC enhancer GRCh37_chr13:101196750-101197473 Neighboring gene H3K4me1 hESC enhancer GRCh37_chr13:101197474-101198196 Neighboring gene H3K27ac-H3K4me1 hESC enhancer GRCh37_chr13:101198945-101199623 Neighboring gene H3K27ac-H3K4me1 hESC enhancer GRCh37_chr13:101199624-101200301 Neighboring gene ribosomal protein S26 pseudogene 47 Neighboring gene ATAC-STARR-seq lymphoblastoid silent region 5477 Neighboring gene gamma-glutamylamine cyclotransferase Neighboring gene transmembrane O-mannosyltransferase targeting cadherins 4 Neighboring gene cytochrome c oxidase subunit 5B pseudogene 6

    Genomic regions, transcripts, and products

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    Go to nucleotide: Graphics FASTA GenBank

    Expression

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    • Project title: HPA RNA-seq normal tissues
    • Description: RNA-seq was performed of tissue samples from 95 human individuals representing 27 different tissues in order to determine tissue-specificity of all protein-coding genes
    • BioProject: PRJEB4337
    • Publication: PMID 24309898
    • Analysis date: Wed Apr 4 07:08:55 2018

    Bibliography

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    Related articles in PubMed

    1. Clinical characteristics and mutation analysis of propionic acidemia in Thailand. Vatanavicharn N, et al. World J Pediatr, 2014 Feb. PMID 24464666
    2. Propionic acidemia associated with visual hallucinations. Shuaib T, et al. J Child Neurol, 2012 Jun. PMID 22156789
    3. High frequency of large genomic deletions in the PCCA gene causing propionic acidemia. Desviat LR, et al. Mol Genet Metab, 2009 Apr. PMID 19157943
    4. Characterization of four variant forms of human propionyl-CoA carboxylase expressed in Escherichia coli. Jiang H, et al. J Biol Chem, 2005 Jul 29. PMID 15890657
    5. Functional characterization of PCCA mutations causing propionic acidemia. Clavero S, et al. Biochim Biophys Acta, 2002 Nov 20. PMID 12385775

    See all (111) citations in PubMed

    GeneRIFs: Gene References Into Functions

    What's a GeneRIF?
    1. Differentially expressed genes PCCA, ECHS1, and HADH are potential prognostic biomarkers for gastric cancer.
      Title: Differentially expressed genes PCCA, ECHS1, and HADH are potential prognostic biomarkers for gastric cancer.
    2. A novel small molecule approach for the treatment of propionic and methylmalonic acidemias.
      Title: A novel small molecule approach for the treatment of propionic and methylmalonic acidemias.
    3. Functional Analysis of the PCCA and PCCB Gene Variants Predicted to Affect Splicing.
      Title: Functional Analysis of the PCCA and PCCB Gene Variants Predicted to Affect Splicing.
    4. his work represents a large-scale update on pathogenic mutations in the PCCA and PCCB genes causing Propionic acidemia (PA), and confirms previous reports indicating a major causative role of mutation-induced protein destabilization
      Title: Identification of 34 novel mutations in propionic acidemia: Functional characterization of missense variants and phenotype associations.
    5. The data indicate that amino acid/nucleotide metabolism-related genes OGDH, PPAT and PCCA acquire somatic mutations in microsatellite instability-high gastric cancers and colorectal cancers and that mutational intratumoral heterogeneity may occur in at least some of these tumors.
      Title: Frameshift mutations of OGDH, PPAT and PCCA genes in gastric and colorectal cancers.
    6. The majority of patients had mutations in the PCCA gene (18/25). A total of 26 mutations were noted: 20 in the PCCA gene and 6 in PCCB gene. Seventeen mutations were novel (14 in PCCA and 3 in PCCB). The SNP c.937C>T (p.Arg313Ter), was noted in 9/36 (25%) alleles in the PCCA gene
      Title: Seventeen Novel Mutations in PCCA and PCCB Genes in Indian Propionic Acidemia Patients, and Their Outcomes.
    7. Ten propionic acidemia mutations were confirmed, including 8 affecting the PCCA gene and 2 affecting the PCCB gene
      Title: [Analysis of PCCA and PCCB gene mutations in patients with propionic acidemia].
    8. Two PCCA mutations, c.229C-->T (p.R77W) and c.1262A-->C (p.Q421P), were identified in a PCCA-deficient patient.
      Title: Two frequent mutations associated with the classic form of propionic acidemia in Taiwan.
    9. Mutation analysis of the PCCA gene identified homozygous c.1284+1G>A in patient 1, c.230G>A (p.R77Q) and c.1855C>T (p.R619X) in patient 2, homozygous c.2125T>C (p.S709P) in patient 3, and only one mutant allele, c.231+1G>T in patient 4.
      Title: Clinical characteristics and mutation analysis of propionic acidemia in Thailand.
    10. Thsi study presented that Propionic acidemia(PCCA mutation) associated with visual hallucinations
      Title: Propionic acidemia associated with visual hallucinations.
    Submit: New GeneRIF Correction See all GeneRIFs (21)

    Phenotypes

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    Find tests for this gene in the NIH Genetic Testing Registry (GTR)

    Review eQTL and phenotype association data in this region using PheGenI

    Associated conditions

    Description Tests
    Propionic acidemia
    MedGen: C0268579 OMIM: 606054 GeneReviews: Propionic Acidemia
    		Compare labs

    EBI GWAS Catalog

    Description
    Genome-wide meta-analyses of multiancestry cohorts identify multiple new susceptibility loci for refractive error and myopia.
    EBI GWAS Catalog

    Variation

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    See variants in ClinVar

    See studies and variants in dbVar

    See Variation Viewer (GRCh37.p13)

    See Variation Viewer (GRCh38)

    Genotypes

    Pathways from PubChem

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    Interactions

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    Products Interactant Other Gene Complex Source Pubs Description

    General gene information

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    Markers

    Homology

    Gene Ontology Provided by GOA

    Function Evidence Code Pubs
    enables ATP binding IEA
    Inferred from Electronic Annotation
    more info
    		  
    enables biotin binding TAS
    Traceable Author Statement
    more info
    		 PubMed 
    enables enzyme binding IPI
    Inferred from Physical Interaction
    more info
    		 PubMed 
    enables metal ion binding IEA
    Inferred from Electronic Annotation
    more info
    		  
    enables propionyl-CoA carboxylase activity IBA
    Inferred from Biological aspect of Ancestor
    more info
    		  
    enables propionyl-CoA carboxylase activity IDA
    Inferred from Direct Assay
    more info
    		 PubMed 
    enables propionyl-CoA carboxylase activity IMP
    Inferred from Mutant Phenotype
    more info
    		 PubMed 
    enables protein binding IPI
    Inferred from Physical Interaction
    more info
    		 PubMed 
    Process Evidence Code Pubs
    involved_in branched-chain amino acid metabolic process NAS
    Non-traceable Author Statement
    more info
    		 PubMed 
    involved_in fatty acid metabolic process NAS
    Non-traceable Author Statement
    more info
    		 PubMed 
    involved_in short-chain fatty acid catabolic process IC
    Inferred by Curator
    more info
    		 PubMed 
    Component Evidence Code Pubs
    part_of catalytic complex IPI
    Inferred from Physical Interaction
    more info
    		 PubMed 
    located_in cytosol TAS
    Traceable Author Statement
    more info
    		  
    located_in mitochondrial matrix IDA
    Inferred from Direct Assay
    more info
    		 PubMed 
    located_in mitochondrial matrix NAS
    Non-traceable Author Statement
    more info
    		 PubMed 
    located_in mitochondrial matrix TAS
    Traceable Author Statement
    more info
    		  
    is_active_in mitochondrion IBA
    Inferred from Biological aspect of Ancestor
    more info
    		  
    located_in mitochondrion IDA
    Inferred from Direct Assay
    more info
    		  

    General protein information

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    Preferred Names
    propionyl-CoA carboxylase alpha chain, mitochondrial
    Names
    PCCase alpha subunit
    pccA complementation group
    propanoyl-CoA:carbon dioxide ligase alpha subunit
    propionyl CoA carboxylase, alpha polypeptide
    propionyl Coenzyme A carboxylase, alpha polypeptide
    propionyl-CoA carboxylase alpha subunit
    NP_000273.2
    NP_001121164.1
    NP_001171475.1
    NP_001339534.1
    NP_001339535.1
    NP_001339536.1
    NP_001339537.1
    NP_001339538.1
    NP_001339539.1
    NP_001339540.1
    NP_001339541.1
    XP_011519395.1
    XP_016876094.1
    XP_016876095.1
    XP_016876096.1
    XP_016876098.1
    XP_016876100.1
    XP_016876101.1
    XP_016876102.1
    XP_016876104.1
    XP_016876105.1
    XP_047286326.1
    XP_047286327.1
    XP_047286328.1
    XP_047286329.1
    XP_047286330.1
    XP_047286331.1
    XP_047286332.1
    XP_047286333.1
    XP_047286334.1
    XP_054230547.1
    XP_054230548.1
    XP_054230549.1
    XP_054230550.1
    XP_054230551.1
    XP_054230552.1
    XP_054230553.1
    XP_054230554.1
    XP_054230556.1
    XP_054230557.1
    XP_054230558.1
    XP_054230559.1
    XP_054230560.1
    XP_054230561.1
    XP_054230562.1
    XP_054230563.1
    XP_054230564.1
    XP_054230565.1

    NCBI Reference Sequences (RefSeq)

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    NEW Try the new Transcript table

    RefSeqs maintained independently of Annotated Genomes

    These reference sequences exist independently of genome builds. Explain

    These reference sequences are curated independently of the genome annotation cycle, so their versions may not match the RefSeq versions in the current genome build. Identify version mismatches by comparing the version of the RefSeq in this section to the one reported in Genomic regions, transcripts, and products above.

    Genomic

    1. NG_008768.1 RefSeqGene

      Range
      5011..446353
      Download
      GenBank, FASTA, Sequence Viewer (Graphics)

    mRNA and Protein(s)

    1. NM_000282.4 → NP_000273.2  propionyl-CoA carboxylase alpha chain, mitochondrial isoform a precursor

      See identical proteins and their annotated locations for NP_000273.2

      Status: REVIEWED

      Description
      Transcript Variant: This variant (1) encodes the longest isoform (a).
      Source sequence(s)
      AF385926, BC000140
      Consensus CDS
      CCDS9496.2
      UniProtKB/Swiss-Prot
      B4DKY8, B4DPF9, C9JPQ8, P05165, Q15979, Q8WXQ7
      UniProtKB/TrEMBL
      B2RDE0
      Related
      ENSP00000365462.1, ENST00000376285.6
      Conserved Domains (5) summary
      cd06850
      Location:661 → 727
      biotinyl_domain; The biotinyl-domain or biotin carboxyl carrier protein (BCCP) domain is present in all biotin-dependent enzymes, such as acetyl-CoA carboxylase, pyruvate carboxylase, propionyl-CoA carboxylase, methylcrotonyl-CoA carboxylase, geranyl-CoA carboxylase, ...
      smart00878
      Location:397 → 504
      Biotin_carb_C; Biotin carboxylase C-terminal domain
      COG4770
      Location:63 → 728
      PccA; Acetyl/propionyl-CoA carboxylase, alpha subunit [Lipid transport and metabolism]
      pfam00289
      Location:63 → 171
      CPSase_L_chain; Carbamoyl-phosphate synthase L chain, N-terminal domain
      pfam02786
      Location:176 → 383
      CPSase_L_D2; Carbamoyl-phosphate synthase L chain, ATP binding domain

    2. NM_001127692.3 → NP_001121164.1  propionyl-CoA carboxylase alpha chain, mitochondrial isoform b

      Status: REVIEWED

      Description
      Transcript Variant: This variant (2) lacks an alternate in-frame exon compared to variant 1. The resulting isoform (b) has the same N- and C-termini but is shorter compared to NM_000282.3.
      Source sequence(s)
      AF385926, BC000140, DA781564
      Consensus CDS
      CCDS45065.1
      UniProtKB/TrEMBL
      B2RDE0
      Related
      ENSP00000365463.4, ENST00000376286.8
      Conserved Domains (5) summary
      cd06850
      Location:635 → 701
      biotinyl_domain; The biotinyl-domain or biotin carboxyl carrier protein (BCCP) domain is present in all biotin-dependent enzymes, such as acetyl-CoA carboxylase, pyruvate carboxylase, propionyl-CoA carboxylase, methylcrotonyl-CoA carboxylase, geranyl-CoA carboxylase, ...
      smart00878
      Location:371 → 478
      Biotin_carb_C; Biotin carboxylase C-terminal domain
      COG4770
      Location:37 → 702
      PccA; Acetyl/propionyl-CoA carboxylase, alpha subunit [Lipid transport and metabolism]
      pfam00289
      Location:37 → 145
      CPSase_L_chain; Carbamoyl-phosphate synthase L chain, N-terminal domain
      pfam02786
      Location:150 → 357
      CPSase_L_D2; Carbamoyl-phosphate synthase L chain, ATP binding domain

    3. NM_001178004.2 → NP_001171475.1  propionyl-CoA carboxylase alpha chain, mitochondrial isoform c precursor

      Status: REVIEWED

      Description
      Transcript Variant: This variant (3) lacks an alternate in-frame exon in the 3' coding region compared to variant 1. The resulting protein (isoform c) is shorter compared to isoform a.
      Source sequence(s)
      AK298318, AL356575, CB851764
      Consensus CDS
      CCDS53878.1
      UniProtKB/TrEMBL
      B2RDE0
      Related
      ENSP00000365456.3, ENST00000376279.7
      Conserved Domains (5) summary
      cd06850
      Location:631 → 680
      biotinyl_domain; The biotinyl-domain or biotin carboxyl carrier protein (BCCP) domain is present in all biotin-dependent enzymes, such as acetyl-CoA carboxylase, pyruvate carboxylase, propionyl-CoA carboxylase, methylcrotonyl-CoA carboxylase, geranyl-CoA carboxylase, ...
      smart00878
      Location:397 → 504
      Biotin_carb_C; Biotin carboxylase C-terminal domain
      COG4770
      Location:63 → 681
      PccA; Acetyl/propionyl-CoA carboxylase, alpha subunit [Lipid transport and metabolism]
      pfam00289
      Location:63 → 171
      CPSase_L_chain; Carbamoyl-phosphate synthase L chain, N-terminal domain
      pfam02786
      Location:176 → 383
      CPSase_L_D2; Carbamoyl-phosphate synthase L chain, ATP binding domain

    4. NM_001352605.2 → NP_001339534.1  propionyl-CoA carboxylase alpha chain, mitochondrial isoform d precursor

      Status: REVIEWED

      Source sequence(s)
      AL136526, AL353697, AL355338, AL356575
      UniProtKB/TrEMBL
      B2RDE0
      Conserved Domains (1) summary
      COG4770
      Location:63 → 710
      PccA; Acetyl/propionyl-CoA carboxylase, alpha subunit [Lipid transport and metabolism]

    5. NM_001352606.2 → NP_001339535.1  propionyl-CoA carboxylase alpha chain, mitochondrial isoform e precursor

      Status: REVIEWED

      Source sequence(s)
      AL136526, AL353697, AL355338, AL356575
      UniProtKB/TrEMBL
      A0A1B0GU58
      Related
      ENSP00000489974.1, ENST00000637657.1
      Conserved Domains (1) summary
      cl27361
      Location:63 → 680
      Biotin_carb_C; Biotin carboxylase C-terminal domain

    6. NM_001352607.2 → NP_001339536.1  propionyl-CoA carboxylase alpha chain, mitochondrial isoform f

      Status: REVIEWED

      Source sequence(s)
      AL136526, AL353697, AL355338, AL356575
      UniProtKB/TrEMBL
      B2RDE0
      Conserved Domains (1) summary
      cl27361
      Location:37 → 655
      Biotin_carb_C; Biotin carboxylase C-terminal domain

    7. NM_001352608.2 → NP_001339537.1  propionyl-CoA carboxylase alpha chain, mitochondrial isoform g

      Status: REVIEWED

      Source sequence(s)
      AL136526, AL353697, AL355338, AL356575
      UniProtKB/TrEMBL
      A0A1B0GU58
      Conserved Domains (1) summary
      cl27361
      Location:37 → 654
      Biotin_carb_C; Biotin carboxylase C-terminal domain

    8. NM_001352609.2 → NP_001339538.1  propionyl-CoA carboxylase alpha chain, mitochondrial isoform h precursor

      Status: REVIEWED

      Source sequence(s)
      AL136526, AL353697, AL355338, AL356575
      UniProtKB/TrEMBL
      B2RDE0
      Conserved Domains (1) summary
      cl27361
      Location:63 → 592
      Biotin_carb_C; Biotin carboxylase C-terminal domain

    9. NM_001352610.2 → NP_001339539.1  propionyl-CoA carboxylase alpha chain, mitochondrial isoform i

      Status: REVIEWED

      Source sequence(s)
      AL136526, AL353697, AL355338, AL356575
      Conserved Domains (1) summary
      cl27361
      Location:1 → 413
      Biotin_carb_C; Biotin carboxylase C-terminal domain

    10. NM_001352611.2 → NP_001339540.1  propionyl-CoA carboxylase alpha chain, mitochondrial isoform j

      Status: REVIEWED

      Source sequence(s)
      AL136526, AL353697, AL355338, AL356575
      Conserved Domains (1) summary
      cl27361
      Location:1 → 395
      Biotin_carb_C; Biotin carboxylase C-terminal domain

    11. NM_001352612.2 → NP_001339541.1  propionyl-CoA carboxylase alpha chain, mitochondrial isoform k

      Status: REVIEWED

      Source sequence(s)
      AL136526, AL353697, AL355338, AL356575
      Conserved Domains (1) summary
      cl27361
      Location:1 → 365
      Biotin_carb_C; Biotin carboxylase C-terminal domain

    RNA

    1. NR_148027.2 RNA Sequence

      Status: REVIEWED

      Source sequence(s)
      AL136526, AL353697, AL355338, AL356575

    2. NR_148028.2 RNA Sequence

      Status: REVIEWED

      Source sequence(s)
      AL136526, AL353697, AL355338, AL356575

    3. NR_148029.2 RNA Sequence

      Status: REVIEWED

      Source sequence(s)
      AL136526, AL353697, AL355338, AL356575

    4. NR_148030.2 RNA Sequence

      Status: REVIEWED

      Source sequence(s)
      AL136526, AL353697, AL355338, AL356575

    5. NR_148031.2 RNA Sequence

      Status: REVIEWED

      Source sequence(s)
      AL136526, AL353697, AL355338, AL356575

    RefSeqs of Annotated Genomes: GCF_000001405.40-RS_2023_10

    The following sections contain reference sequences that belong to a specific genome build. Explain

    Reference GRCh38.p14 Primary Assembly

    Genomic

    1. NC_000013.11 Reference GRCh38.p14 Primary Assembly

      Range
      100089093..100530435
      Download
      GenBank, FASTA, Sequence Viewer (Graphics)

    mRNA and Protein(s)

    1. XM_017020609.2 → XP_016876098.1  propionyl-CoA carboxylase alpha chain, mitochondrial isoform X5

      UniProtKB/TrEMBL
      B2RDE0
      Conserved Domains (5) summary
      cd06850
      Location:628 → 694
      biotinyl_domain; The biotinyl-domain or biotin carboxyl carrier protein (BCCP) domain is present in all biotin-dependent enzymes, such as acetyl-CoA carboxylase, pyruvate carboxylase, propionyl-CoA carboxylase, methylcrotonyl-CoA carboxylase, geranyl-CoA carboxylase, ...
      smart00878
      Location:364 → 471
      Biotin_carb_C; Biotin carboxylase C-terminal domain
      COG4770
      Location:30 → 695
      PccA; Acetyl/propionyl-CoA carboxylase, alpha subunit [Lipid transport and metabolism]
      pfam00289
      Location:30 → 138
      CPSase_L_chain; Carbamoyl-phosphate synthase L chain, N-terminal domain
      pfam02786
      Location:143 → 350
      CPSase_L_D2; Carbamoyl-phosphate synthase L chain, ATP binding domain

    2. XM_047430373.1 → XP_047286329.1  propionyl-CoA carboxylase alpha chain, mitochondrial isoform X12

    3. XM_017020613.2 → XP_016876102.1  propionyl-CoA carboxylase alpha chain, mitochondrial isoform X11

      UniProtKB/TrEMBL
      B2RDE0

    4. XM_047430371.1 → XP_047286327.1  propionyl-CoA carboxylase alpha chain, mitochondrial isoform X7

    5. XM_017020612.2 → XP_016876101.1  propionyl-CoA carboxylase alpha chain, mitochondrial isoform X9

      UniProtKB/TrEMBL
      B2RDE0

    6. XM_017020611.2 → XP_016876100.1  propionyl-CoA carboxylase alpha chain, mitochondrial isoform X8

      UniProtKB/TrEMBL
      B2RDE0

    7. XM_011521093.2 → XP_011519395.1  propionyl-CoA carboxylase alpha chain, mitochondrial isoform X4

      UniProtKB/TrEMBL
      B2RDE0
      Conserved Domains (4) summary
      smart00878
      Location:397 → 504
      Biotin_carb_C; Biotin carboxylase C-terminal domain
      PRK08591
      Location:62 → 504
      PRK08591; acetyl-CoA carboxylase biotin carboxylase subunit; Validated
      pfam00289
      Location:63 → 171
      CPSase_L_chain; Carbamoyl-phosphate synthase L chain, N-terminal domain
      pfam02786
      Location:176 → 383
      CPSase_L_D2; Carbamoyl-phosphate synthase L chain, ATP binding domain

    8. XM_047430372.1 → XP_047286328.1  propionyl-CoA carboxylase alpha chain, mitochondrial isoform X10

    9. XM_047430375.1 → XP_047286331.1  propionyl-CoA carboxylase alpha chain, mitochondrial isoform X15

    10. XM_017020607.2 → XP_016876096.1  propionyl-CoA carboxylase alpha chain, mitochondrial isoform X3

      UniProtKB/TrEMBL
      B2RDE0

    11. XM_017020605.2 → XP_016876094.1  propionyl-CoA carboxylase alpha chain, mitochondrial isoform X1

    12. XM_017020606.2 → XP_016876095.1  propionyl-CoA carboxylase alpha chain, mitochondrial isoform X2

    13. XM_047430374.1 → XP_047286330.1  propionyl-CoA carboxylase alpha chain, mitochondrial isoform X13

    14. XM_017020615.2 → XP_016876104.1  propionyl-CoA carboxylase alpha chain, mitochondrial isoform X14

    15. XM_017020616.2 → XP_016876105.1  propionyl-CoA carboxylase alpha chain, mitochondrial isoform X16

    16. XM_047430377.1 → XP_047286333.1  propionyl-CoA carboxylase alpha chain, mitochondrial isoform X18

      Related
      ENSP00000490843.1, ENST00000636475.1

    17. XM_047430370.1 → XP_047286326.1  propionyl-CoA carboxylase alpha chain, mitochondrial isoform X6

    18. XM_047430376.1 → XP_047286332.1  propionyl-CoA carboxylase alpha chain, mitochondrial isoform X17

    19. XM_047430378.1 → XP_047286334.1  propionyl-CoA carboxylase alpha chain, mitochondrial isoform X19

    RNA

    1. XR_007063681.1 RNA Sequence

    Alternate T2T-CHM13v2.0

    Genomic

    1. NC_060937.1 Alternate T2T-CHM13v2.0

      Range
      99302579..99745721
      Download
      GenBank, FASTA, Sequence Viewer (Graphics)

    mRNA and Protein(s)

    1. XM_054374576.1 → XP_054230551.1  propionyl-CoA carboxylase alpha chain, mitochondrial isoform X5

    2. XM_054374583.1 → XP_054230558.1  propionyl-CoA carboxylase alpha chain, mitochondrial isoform X12

    3. XM_054374574.1 → XP_054230549.1  propionyl-CoA carboxylase alpha chain, mitochondrial isoform X3

    4. XM_054374582.1 → XP_054230557.1  propionyl-CoA carboxylase alpha chain, mitochondrial isoform X11

    5. XM_054374578.1 → XP_054230553.1  propionyl-CoA carboxylase alpha chain, mitochondrial isoform X7

    6. XM_054374579.1 → XP_054230554.1  propionyl-CoA carboxylase alpha chain, mitochondrial isoform X8

    7. XM_054374575.1 → XP_054230550.1  propionyl-CoA carboxylase alpha chain, mitochondrial isoform X4

    8. XM_054374581.1 → XP_054230556.1  propionyl-CoA carboxylase alpha chain, mitochondrial isoform X10

    9. XM_054374586.1 → XP_054230561.1  propionyl-CoA carboxylase alpha chain, mitochondrial isoform X15

    10. XM_054374572.1 → XP_054230547.1  propionyl-CoA carboxylase alpha chain, mitochondrial isoform X1

    11. XM_054374573.1 → XP_054230548.1  propionyl-CoA carboxylase alpha chain, mitochondrial isoform X2

    12. XM_054374584.1 → XP_054230559.1  propionyl-CoA carboxylase alpha chain, mitochondrial isoform X13

    13. XM_054374585.1 → XP_054230560.1  propionyl-CoA carboxylase alpha chain, mitochondrial isoform X14

    14. XM_054374587.1 → XP_054230562.1  propionyl-CoA carboxylase alpha chain, mitochondrial isoform X16

    15. XM_054374589.1 → XP_054230564.1  propionyl-CoA carboxylase alpha chain, mitochondrial isoform X18

    16. XM_054374577.1 → XP_054230552.1  propionyl-CoA carboxylase alpha chain, mitochondrial isoform X6

    17. XM_054374588.1 → XP_054230563.1  propionyl-CoA carboxylase alpha chain, mitochondrial isoform X17

    18. XM_054374590.1 → XP_054230565.1  propionyl-CoA carboxylase alpha chain, mitochondrial isoform X19

    RNA

    1. XR_008488765.1 RNA Sequence

    2. XR_008488764.1 RNA Sequence

    3. XR_008488763.1 RNA Sequence

    Nucleotide Protein
    Heading Accession and Version
    Protein Accession Links
    GenPept Link UniProtKB Link
    P05165.4 GenPept UniProtKB/Swiss-Prot:P05165

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