LOCUS       NM_000282               2484 bp    mRNA    linear   PRI 10-DEC-2024
DEFINITION  Homo sapiens propionyl-CoA carboxylase subunit alpha (PCCA),
            transcript variant 1, mRNA; nuclear gene for mitochondrial product.
ACCESSION   NM_000282
VERSION     NM_000282.4
KEYWORDS    RefSeq; MANE Select.
SOURCE      Homo sapiens (human)
  ORGANISM  Homo sapiens
            Eukaryota; Metazoa; Chordata; Craniata; Vertebrata; Euteleostomi;
            Mammalia; Eutheria; Euarchontoglires; Primates; Haplorrhini;
            Catarrhini; Hominidae; Homo.
REFERENCE   1  (bases 1 to 2484)
  AUTHORS   Wang,J., Cui,M., Liu,Y., Chen,M., Xu,J., Xia,J., Sun,J., Jiang,L.,
            Fang,W., Song,H. and Cheng,C.
  TITLE     The mitochondrial carboxylase PCCA interacts with Listeria
            monocytogenes phospholipase PlcB to modulate bacterial survival
  JOURNAL   Appl Environ Microbiol 90 (6), e0213523 (2024)
   PUBMED   38727222
  REMARK    GeneRIF: The mitochondrial carboxylase PCCA interacts with Listeria
            monocytogenes phospholipase PlcB to modulate bacterial survival.
REFERENCE   2  (bases 1 to 2484)
  AUTHORS   Morgenstern,M., Peikert,C.D., Lubbert,P., Suppanz,I., Klemm,C.,
            Alka,O., Steiert,C., Naumenko,N., Schendzielorz,A., Melchionda,L.,
            Muhlhauser,W.W.D., Knapp,B., Busch,J.D., Stiller,S.B.,
            Dannenmaier,S., Lindau,C., Licheva,M., Eickhorst,C., Galbusera,R.,
            Zerbes,R.M., Ryan,M.T., Kraft,C., Kozjak-Pavlovic,V., Drepper,F.,
            Dennerlein,S., Oeljeklaus,S., Pfanner,N., Wiedemann,N. and
            Warscheid,B.
  TITLE     Quantitative high-confidence human mitochondrial proteome and its
            dynamics in cellular context
  JOURNAL   Cell Metab 33 (12), 2464-2483 (2021)
   PUBMED   34800366
REFERENCE   3  (bases 1 to 2484)
  AUTHORS   Huttlin,E.L., Bruckner,R.J., Navarrete-Perea,J., Cannon,J.R.,
            Baltier,K., Gebreab,F., Gygi,M.P., Thornock,A., Zarraga,G., Tam,S.,
            Szpyt,J., Gassaway,B.M., Panov,A., Parzen,H., Fu,S., Golbazi,A.,
            Maenpaa,E., Stricker,K., Guha Thakurta,S., Zhang,T., Rad,R.,
            Pan,J., Nusinow,D.P., Paulo,J.A., Schweppe,D.K., Vaites,L.P.,
            Harper,J.W. and Gygi,S.P.
  TITLE     Dual proteome-scale networks reveal cell-specific remodeling of the
            human interactome
  JOURNAL   Cell 184 (11), 3022-3040 (2021)
   PUBMED   33961781
REFERENCE   4  (bases 1 to 2484)
  AUTHORS   Bychkov,I., Galushkin,A., Filatova,A., Nekrasov,A., Kurkina,M.,
            Baydakova,G., Ilyushkina,A., Skoblov,M. and Zakharova,E.
  TITLE     Functional Analysis of the PCCA and PCCB Gene Variants Predicted to
            Affect Splicing
  JOURNAL   Int J Mol Sci 22 (8), 4154 (2021)
   PUBMED   33923806
  REMARK    GeneRIF: Functional Analysis of the PCCA and PCCB Gene Variants
            Predicted to Affect Splicing.
            Publication Status: Online-Only
REFERENCE   5  (bases 1 to 2484)
  AUTHORS   Du,Z., Zhang,X., Gao,W. and Yang,J.
  TITLE     Differentially expressed genes PCCA, ECHS1, and HADH are potential
            prognostic biomarkers for gastric cancer
  JOURNAL   Sci Prog 104 (2), 368504211011344 (2021)
   PUBMED   33881965
  REMARK    GeneRIF: Differentially expressed genes PCCA, ECHS1, and HADH are
            potential prognostic biomarkers for gastric cancer.
REFERENCE   6  (bases 1 to 2484)
  AUTHORS   Galarreta Aima,C.I., Shchelochkov,O.A., Jerves Serrano,T. and
            Venditti,C.P.
  TITLE     Propionic Acidemia
  JOURNAL   (in) Adam MP, Feldman J, Mirzaa GM, Pagon RA, Wallace SE and
            Amemiya A (Eds.);
            GENEREVIEWS(R);
            (1993)
   PUBMED   22593918
REFERENCE   7  (bases 1 to 2484)
  AUTHORS   Kennerknecht,I., Klett,C. and Hameister,H.
  TITLE     Assignment of the human gene propionyl coenzyme A carboxylase,
            alpha-chain, (PCCA) to chromosome 13q32 by in situ hybridization
  JOURNAL   Genomics 14 (2), 550-551 (1992)
   PUBMED   1427880
REFERENCE   8  (bases 1 to 2484)
  AUTHORS   Lamhonwah,A.M., Mahuran,D. and Gravel,R.A.
  TITLE     Human mitochondrial propionyl-CoA carboxylase: localization of the
            N-terminus of the pro- and mature alpha chains in the deduced
            primary sequence of a full-length cDNA
  JOURNAL   Nucleic Acids Res 17 (11), 4396 (1989)
   PUBMED   2740237
REFERENCE   9  (bases 1 to 2484)
  AUTHORS   Lamhonwah,A.M., Quan,F. and Gravel,R.A.
  TITLE     Sequence homology around the biotin-binding site of human
            propionyl-CoA carboxylase and pyruvate carboxylase
  JOURNAL   Arch Biochem Biophys 254 (2), 631-636 (1987)
   PUBMED   3555348
REFERENCE   10 (bases 1 to 2484)
  AUTHORS   Lamhonwah,A.M., Barankiewicz,T.J., Willard,H.F., Mahuran,D.J.,
            Quan,F. and Gravel,R.A.
  TITLE     Isolation of cDNA clones coding for the alpha and beta chains of
            human propionyl-CoA carboxylase: chromosomal assignments and DNA
            polymorphisms associated with PCCA and PCCB genes
  JOURNAL   Proc Natl Acad Sci U S A 83 (13), 4864-4868 (1986)
   PUBMED   3460076
COMMENT     REVIEWED REFSEQ: This record has been curated by NCBI staff. The
            reference sequence was derived from AF385926.1 and BC000140.2.
            This sequence is a reference standard in the RefSeqGene project.
            
            On Nov 22, 2018 this sequence version replaced NM_000282.3.
            
            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].
            
            Transcript Variant: This variant (1) encodes the longest isoform
            (a).
            
            Sequence Note: This RefSeq record was created from transcript and
            genomic sequence data to make the sequence consistent with the
            reference genome assembly. The genomic coordinates used for the
            transcript record were based on transcript alignments.
            
            Publication Note:  This RefSeq record includes a subset of the
            publications that are available for this gene. Please see the Gene
            record to access additional publications.
            
            ##Evidence-Data-START##
            Transcript exon combination :: SRR1803612.106251.1, AF385926.1
                                           [ECO:0000332]
            RNAseq introns              :: single sample supports all introns
                                           SAMEA1966682, SAMEA1968189
                                           [ECO:0000348]
            ##Evidence-Data-END##
            
            ##RefSeq-Attributes-START##
            gene product(s) localized to mito. :: reported by MitoCarta
            MANE Ensembl match                 :: ENST00000376285.6/
                                                  ENSP00000365462.1
            RefSeq Select criteria             :: based on conservation,
                                                  expression
            ##RefSeq-Attributes-END##
            COMPLETENESS: full length.
PRIMARY     REFSEQ_SPAN         PRIMARY_IDENTIFIER PRIMARY_SPAN        COMP
            1-2393              AF385926.1         11-2403
            2394-2484           BC000140.2         2344-2434
FEATURES             Location/Qualifiers
     source          1..2484
                     /organism="Homo sapiens"
                     /mol_type="mRNA"
                     /db_xref="taxon:9606"
                     /chromosome="13"
                     /map="13q32.3"
     gene            1..2484
                     /gene="PCCA"
                     /note="propionyl-CoA carboxylase subunit alpha"
                     /db_xref="GeneID:5095"
                     /db_xref="HGNC:HGNC:8653"
                     /db_xref="MIM:232000"
     exon            1..133
                     /gene="PCCA"
                     /inference="alignment:Splign:2.1.0"
     CDS             29..2215
                     /gene="PCCA"
                     /EC_number="6.4.1.3"
                     /note="isoform a precursor is encoded by transcript
                     variant 1; propionyl Coenzyme A carboxylase, alpha
                     polypeptide; propionyl-CoA carboxylase alpha chain,
                     mitochondrial; PCCase alpha subunit; propanoyl-CoA:carbon
                     dioxide ligase alpha subunit; pccA complementation group;
                     propionyl-CoA carboxylase alpha subunit; propionyl CoA
                     carboxylase, alpha polypeptide"
                     /codon_start=1
                     /product="propionyl-CoA carboxylase alpha chain,
                     mitochondrial isoform a precursor"
                     /protein_id="NP_000273.2"
                     /db_xref="CCDS:CCDS9496.2"
                     /db_xref="GeneID:5095"
                     /db_xref="HGNC:HGNC:8653"
                     /db_xref="MIM:232000"
                     /translation="MAGFWVGTAPLVAAGRRGRWPPQQLMLSAALRTLKHVLYYSRQC
                     LMVSRNLGSVGYDPNEKTFDKILVANRGEIACRVIRTCKKMGIKTVAIHSDVDASSVH
                     VKMADEAVCVGPAPTSKSYLNMDAIMEAIKKTRAQAVHPGYGFLSENKEFARCLAAED
                     VVFIGPDTHAIQAMGDKIESKLLAKKAEVNTIPGFDGVVKDAEEAVRIAREIGYPVMI
                     KASAGGGGKGMRIAWDDEETRDGFRLSSQEAASSFGDDRLLIEKFIDNPRHIEIQVLG
                     DKHGNALWLNERECSIQRRNQKVVEEAPSIFLDAETRRAMGEQAVALARAVKYSSAGT
                     VEFLVDSKKNFYFLEMNTRLQVEHPVTECITGLDLVQEMIRVAKGYPLRHKQADIRIN
                     GWAVECRVYAEDPYKSFGLPSIGRLSQYQEPLHLPGVRVDSGIQPGSDISIYYDPMIS
                     KLITYGSDRTEALKRMADALDNYVIRGVTHNIALLREVIINSRFVKGDISTKFLSDVY
                     PDGFKGHMLTKSEKNQLLAIASSLFVAFQLRAQHFQENSRMPVIKPDIANWELSVKLH
                     DKVHTVVASNNGSVFSVEVDGSKLNVTSTWNLASPLLSVSVDGTQRTVQCLSREAGGN
                     MSIQFLGTVYKVNILTRLAAELNKFMLEKVTEDTSSVLRSPMPGVVVAVSVKPGDAVA
                     EGQEICVIEAMKMQNSMTAGKTGTVKSVHCQAGDTVGEGDLLVELE"
     exon            134..211
                     /gene="PCCA"
                     /inference="alignment:Splign:2.1.0"
     exon            212..259
                     /gene="PCCA"
                     /inference="alignment:Splign:2.1.0"
     exon            260..328
                     /gene="PCCA"
                     /inference="alignment:Splign:2.1.0"
     exon            329..442
                     /gene="PCCA"
                     /inference="alignment:Splign:2.1.0"
     exon            443..496
                     /gene="PCCA"
                     /inference="alignment:Splign:2.1.0"
     exon            497..628
                     /gene="PCCA"
                     /inference="alignment:Splign:2.1.0"
     exon            629..665
                     /gene="PCCA"
                     /inference="alignment:Splign:2.1.0"
     exon            666..744
                     /gene="PCCA"
                     /inference="alignment:Splign:2.1.0"
     exon            745..847
                     /gene="PCCA"
                     /inference="alignment:Splign:2.1.0"
     exon            848..942
                     /gene="PCCA"
                     /inference="alignment:Splign:2.1.0"
     exon            943..1093
                     /gene="PCCA"
                     /inference="alignment:Splign:2.1.0"
     exon            1094..1237
                     /gene="PCCA"
                     /inference="alignment:Splign:2.1.0"
     exon            1238..1312
                     /gene="PCCA"
                     /inference="alignment:Splign:2.1.0"
     exon            1313..1381
                     /gene="PCCA"
                     /inference="alignment:Splign:2.1.0"
     exon            1382..1457
                     /gene="PCCA"
                     /inference="alignment:Splign:2.1.0"
     exon            1458..1568
                     /gene="PCCA"
                     /inference="alignment:Splign:2.1.0"
     exon            1569..1671
                     /gene="PCCA"
                     /inference="alignment:Splign:2.1.0"
     exon            1672..1774
                     /gene="PCCA"
                     /inference="alignment:Splign:2.1.0"
     exon            1775..1873
                     /gene="PCCA"
                     /inference="alignment:Splign:2.1.0"
     exon            1874..1927
                     /gene="PCCA"
                     /inference="alignment:Splign:2.1.0"
     exon            1928..2068
                     /gene="PCCA"
                     /inference="alignment:Splign:2.1.0"
     exon            2069..2146
                     /gene="PCCA"
                     /inference="alignment:Splign:2.1.0"
     exon            2147..2484
                     /gene="PCCA"
                     /inference="alignment:Splign:2.1.0"
     regulatory      2461..2466
                     /regulatory_class="polyA_signal_sequence"
                     /gene="PCCA"
                     /note="hexamer: AATAAA"
     polyA_site      2484
                     /gene="PCCA"
                     /note="major polyA site"
ORIGIN      
        1 agcagagggg cggtctgcgg ggacaacaat ggcggggttc tgggtcggga cagcaccgct
       61 ggtcgctgcc ggacggcgtg ggcggtggcc gccgcagcag ctgatgctga gcgcggcgct
      121 gcggaccctg aagcatgttc tgtactattc aagacagtgc ttaatggtgt cccgtaatct
      181 tggttcagtg ggatatgatc ctaatgaaaa aacttttgat aaaattcttg ttgctaatag
      241 aggagaaatt gcatgtcggg ttattagaac ttgcaagaag atgggcatta agacagttgc
      301 catccacagt gatgttgatg ctagttctgt tcatgtgaaa atggcggatg aggctgtctg
      361 tgttggccca gctcccacca gtaaaagcta cctcaacatg gatgccatca tggaagccat
      421 taagaaaacc agggcccaag ctgtacatcc aggttatgga ttcctttcag aaaacaaaga
      481 atttgccaga tgtttggcag cagaagatgt cgttttcatt ggacctgaca cacatgctat
      541 tcaagccatg ggcgacaaga ttgaaagcaa attattagct aagaaagcag aggttaatac
      601 aatccctggc tttgatggag tagtcaagga tgcagaagaa gctgtcagaa ttgcaaggga
      661 aattggctac cctgtcatga tcaaggcctc agcaggtggt ggtgggaaag gcatgcgcat
      721 tgcttgggat gatgaagaga ccagggatgg ttttagattg tcatctcaag aagctgcttc
      781 tagttttggc gatgatagac tactaataga aaaatttatt gataatcctc gtcatataga
      841 aatccaggtt ctaggtgata aacatgggaa tgctttatgg cttaatgaaa gagagtgctc
      901 aattcagaga agaaatcaga aggtggtgga ggaagcacca agcatttttt tggatgcgga
      961 gactcgaaga gcgatgggag aacaagctgt agctcttgcc agagcagtaa aatattcctc
     1021 tgctgggacc gtggagttcc ttgtggactc taagaagaat ttttatttct tggaaatgaa
     1081 tacaagactc caggttgagc atcctgtcac agaatgcatt actggcctgg acctagtcca
     1141 ggaaatgatc cgtgttgcta agggctaccc tctcaggcac aaacaagctg atattcgcat
     1201 caacggctgg gcagttgaat gtcgggttta tgctgaggac ccctacaagt cttttggttt
     1261 accatctatt gggagattgt ctcagtacca agaaccgtta catctacctg gtgtccgagt
     1321 ggacagtggc atccaaccag gaagtgatat tagcatttat tatgatccta tgatttcaaa
     1381 actaatcaca tatggctctg atagaactga ggcactgaag agaatggcag atgcactgga
     1441 taactatgtt attcgaggtg ttacacataa tattgcatta cttcgagagg tgataatcaa
     1501 ctcacgcttt gtaaaaggag acatcagcac taaatttctc tccgatgtgt atcctgatgg
     1561 cttcaaagga cacatgctaa ccaagagtga gaagaaccag ttattggcaa tagcatcatc
     1621 attgtttgtg gcattccagt taagagcaca acattttcaa gaaaattcaa gaatgcctgt
     1681 tattaaacca gacatagcca actgggagct ctcagtaaaa ttgcatgata aagttcatac
     1741 cgtagtagca tcaaacaatg ggtcagtgtt ctcggtggaa gttgatgggt cgaaactaaa
     1801 tgtgaccagc acgtggaacc tggcttcgcc cttattgtct gtcagcgttg atggcactca
     1861 gaggactgtc cagtgtcttt ctcgagaagc aggtggaaac atgagcattc agtttcttgg
     1921 tacagtgtac aaggtgaata tcttaaccag acttgccgca gaattgaaca aatttatgct
     1981 ggaaaaagtg actgaggaca caagcagtgt tctgcgttcc ccgatgcccg gagtggtggt
     2041 ggccgtctct gtcaagcctg gagacgcggt agcagaaggt caagaaattt gtgtgattga
     2101 agccatgaaa atgcagaata gtatgacagc tgggaaaact ggcacggtga aatctgtgca
     2161 ctgtcaagct ggagacacag ttggagaagg ggatctgctc gtggagctgg aatgaaggat
     2221 ttataacctt tcagtcatca cccaatttaa ttagccattt gcatgatgct ttcacacaca
     2281 attgattcaa gcattataca ggaacacccc tgtgcagcta cgtttacgtc gtcatttatt
     2341 ccacagagtc aagaccaata ttctgccaaa aaatcaccaa tggaaatttt cattgatata
     2401 aatacttgta catatgattt gtacttctgc tgtgagattc cctagtgtca aaattaaatc
     2461 aataaaactg agcatttgtc taaa
//
