LOCUS       NM_012782               1690 bp    mRNA    linear   ROD 19-SEP-2024
DEFINITION  Rattus norvegicus branched chain keto acid dehydrogenase E1 subunit
            alpha (Bckdha), mRNA; nuclear gene for mitochondrial product.
ACCESSION   NM_012782 XM_341805
VERSION     NM_012782.1
KEYWORDS    RefSeq; RefSeq Select.
SOURCE      Rattus norvegicus (Norway rat)
  ORGANISM  Rattus norvegicus
            Eukaryota; Metazoa; Chordata; Craniata; Vertebrata; Euteleostomi;
            Mammalia; Eutheria; Euarchontoglires; Glires; Rodentia; Myomorpha;
            Muroidea; Muridae; Murinae; Rattus.
REFERENCE   1  (bases 1 to 1690)
  AUTHORS   Ogawa,T., Kouzu,H., Osanami,A., Tatekoshi,Y., Sato,T., Kuno,A.,
            Fujita,Y., Ino,S., Shimizu,M., Toda,Y., Ohwada,W., Yano,T.,
            Tanno,M., Miki,T. and Miura,T.
  TITLE     Downregulation of extramitochondrial BCKDH and its uncoupling from
            AMP deaminase in type 2 diabetic OLETF rat hearts
  JOURNAL   Physiol Rep 11 (4), e15608 (2023)
   PUBMED   36802195
  REMARK    GeneRIF: Downregulation of extramitochondrial BCKDH and its
            uncoupling from AMP deaminase in type 2 diabetic OLETF rat hearts.
REFERENCE   2  (bases 1 to 1690)
  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 1690)
  AUTHORS   Gordeev,S.A., Bykova,T.V., Zubova,S.G., Aksenov,N.D. and
            Pospelova,T.V.
  TITLE     [Antiapoptotic gene bcl-2 prevents cellular senescence program
            reactivation induced by histone deacetylase inhibitor sodium
            butyrate in E1A and cHa-ras transformed rat fibroblasts]
  JOURNAL   Tsitologiia 57 (2), 135-143 (2015)
   PUBMED   26035971
  REMARK    GeneRIF: It has been found that NaBut reduces proliferation rate of
            ERasBcl (E1A, cHa-ras and bcl-2 ) cells significantly weaker than
            of ERas transformed cells
REFERENCE   4  (bases 1 to 1690)
  AUTHORS   Knapik-Czajka,M.
  TITLE     Simvastatin increases liver branched-chain alpha-ketoacid
            dehydrogenase activity in rats fed with low protein diet
  JOURNAL   Toxicology 325, 107-114 (2014)
   PUBMED   25193403
  REMARK    GeneRIF: Simvastatin stimulates liver BCKDH activity and BCAAs
            degradation in rats fed with the low protein diet.
REFERENCE   5  (bases 1 to 1690)
  AUTHORS   Zhao,X., Leon,I.R., Bak,S., Mogensen,M., Wrzesinski,K., Hojlund,K.
            and Jensen,O.N.
  TITLE     Phosphoproteome analysis of functional mitochondria isolated from
            resting human muscle reveals extensive phosphorylation of inner
            membrane protein complexes and enzymes
  JOURNAL   Mol Cell Proteomics 10 (1), M110.000299 (2011)
   PUBMED   20833797
REFERENCE   6  (bases 1 to 1690)
  AUTHORS   Chuang,J.L., Davie,J.R., Chinsky,J.M., Wynn,R.M., Cox,R.P. and
            Chuang,D.T.
  TITLE     Molecular and biochemical basis of intermediate maple syrup urine
            disease. Occurrence of homozygous G245R and F364C mutations at the
            E1 alpha locus of Hispanic-Mexican patients
  JOURNAL   J Clin Invest 95 (3), 954-963 (1995)
   PUBMED   7883996
REFERENCE   7  (bases 1 to 1690)
  AUTHORS   Chicco,A.G., Adibi,S.A., Liu,W.Q., Morris,S.M. Jr. and Paul,H.S.
  TITLE     Regulation of gene expression of branched-chain keto acid
            dehydrogenase complex in primary cultured hepatocytes by
            dexamethasone and a cAMP analog
  JOURNAL   J Biol Chem 269 (30), 19427-19434 (1994)
   PUBMED   8034710
REFERENCE   8  (bases 1 to 1690)
  AUTHORS   Zhang,B., Zhao,Y., Harris,R.A. and Crabb,D.W.
  TITLE     Molecular defects in the E1 alpha subunit of the branched-chain
            alpha-ketoacid dehydrogenase complex that cause maple syrup urine
            disease
  JOURNAL   Mol Biol Med 8 (1), 39-47 (1991)
   PUBMED   1943689
REFERENCE   9  (bases 1 to 1690)
  AUTHORS   Zhang,B., Kuntz,M.J., Goodwin,G.W., Harris,R.A. and Crabb,D.W.
  TITLE     Molecular cloning of a cDNA for the E1 alpha subunit of rat liver
            branched chain alpha-ketoacid dehydrogenase
  JOURNAL   J Biol Chem 262 (31), 15220-15224 (1987)
   PUBMED   2822716
REFERENCE   10 (bases 1 to 1690)
  AUTHORS   Shimomura,Y., Paxton,R., Ozawa,T. and Harris,R.A.
  TITLE     Purification of branched chain alpha-ketoacid dehydrogenase complex
            from rat liver
  JOURNAL   Anal Biochem 163 (1), 74-78 (1987)
   PUBMED   3619032
COMMENT     VALIDATED REFSEQ: This record has undergone validation or
            preliminary review. The reference sequence was derived from
            CB579195.1, J02827.1 and BC089915.1.
            
            On Oct 17, 2005 this sequence version replaced XM_341805.1.
            
            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 :: BC161819.1, J02827.1 [ECO:0000332]
            RNAseq introns              :: single sample supports all introns
                                           SAMEA5760400, SAMEA5760476
                                           [ECO:0000348]
            ##Evidence-Data-END##
            
            ##RefSeq-Attributes-START##
            gene product(s) localized to mito. :: inferred from homology
            RefSeq Select criteria             :: based on conservation,
                                                  expression, longest protein
            ##RefSeq-Attributes-END##
PRIMARY     REFSEQ_SPAN         PRIMARY_IDENTIFIER PRIMARY_SPAN        COMP
            1-16                CB579195.1         7-22
            17-1363             J02827.1           1-1347
            1364-1690           BC089915.1         1011-1337
FEATURES             Location/Qualifiers
     source          1..1690
                     /organism="Rattus norvegicus"
                     /mol_type="mRNA"
                     /db_xref="taxon:10116"
                     /chromosome="1"
                     /map="1q21"
     gene            1..1690
                     /gene="Bckdha"
                     /gene_synonym="BCKDA; E1a"
                     /note="branched chain keto acid dehydrogenase E1 subunit
                     alpha"
                     /db_xref="GeneID:25244"
                     /db_xref="RGD:2196"
     exon            1..109
                     /gene="Bckdha"
                     /gene_synonym="BCKDA; E1a"
                     /inference="alignment:Splign:2.1.0"
     CDS             2..1342
                     /gene="Bckdha"
                     /gene_synonym="BCKDA; E1a"
                     /EC_number="1.2.4.4"
                     /note="branched chain alpha-ketoacid dehydrogenase subunit
                     E1 alpha; BCKDH E1-alpha; branched-chain alpha-keto acid
                     dehydrogenase E1 component alpha chain; BCKDE1A; branched
                     chain keto acid dehydrogenase subunit E1, alpha
                     polypeptide; branched chain ketoacid dehydrogenase E1,
                     alpha polypeptide"
                     /codon_start=1
                     /product="2-oxoisovalerate dehydrogenase subunit alpha,
                     mitochondrial"
                     /protein_id="NP_036914.1"
                     /db_xref="GeneID:25244"
                     /db_xref="RGD:2196"
                     /translation="MAVAMSAAKIWRPSRGLRQAALLLLGRPGARGLARFHPSRQQQQ
                     QFPSLDDKPQFPGASAEFVDKLEFIQPNVISGIPIYRVMDRQGQIINPSEDPHLPQEE
                     VLKLYRSMTLLNTMDRILYESQRQGRISFYMTNYGEEGTHVGSAAALERTDLVFGQYR
                     EAGVLMYRDYPLELFMAQCYGNVSDPGKGRQMPVHYGCKERHFVTISSPLATQIPQAV
                     GAAYAAKRANANQIVICYFGEGAASEGDAHAGFNFAATLECPIIFFCRNNGYAISTPT
                     SEQYRGDGIAARGPGYGIMSIRVDGNDVFAVYNATKEARRRAVAENQPFLIEAMTYRI
                     GHHSTSDDSSAYRSVDEVNYWDKQDHPISRLRQYLLNQGWWDEEQEKAWRKQSRKKVM
                     EAFEQAERKLKPNPSLLFSDVYQEMPAQLRRQQESLARHLQTYGEHYPLDHFDK"
     exon            110..292
                     /gene="Bckdha"
                     /gene_synonym="BCKDA; E1a"
                     /inference="alignment:Splign:2.1.0"
     exon            293..379
                     /gene="Bckdha"
                     /gene_synonym="BCKDA; E1a"
                     /inference="alignment:Splign:2.1.0"
     exon            380..488
                     /gene="Bckdha"
                     /gene_synonym="BCKDA; E1a"
                     /inference="alignment:Splign:2.1.0"
     exon            489..650
                     /gene="Bckdha"
                     /gene_synonym="BCKDA; E1a"
                     /inference="alignment:Splign:2.1.0"
     exon            651..857
                     /gene="Bckdha"
                     /gene_synonym="BCKDA; E1a"
                     /inference="alignment:Splign:2.1.0"
     exon            858..999
                     /gene="Bckdha"
                     /gene_synonym="BCKDA; E1a"
                     /inference="alignment:Splign:2.1.0"
     exon            1000..1171
                     /gene="Bckdha"
                     /gene_synonym="BCKDA; E1a"
                     /inference="alignment:Splign:2.1.0"
     exon            1172..1658
                     /gene="Bckdha"
                     /gene_synonym="BCKDA; E1a"
                     /inference="alignment:Splign:2.1.0"
ORIGIN      
        1 gatggcggta gcgatgtccg cggccaagat ctggaggccg agccgtggcc tgcgccaggc
       61 tgccctcttg ctgttggggc gacctggggc tcggggcttg gctagattcc accccagcag
      121 gcagcagcaa cagcagttcc catccctgga tgacaagccc cagttcccag gggcctctgc
      181 agagtttgta gacaagcttg agttcatcca gcccaatgtc atctccggca tccccatcta
      241 ccgcgtcatg gaccgacagg gccagatcat caaccccagt gaagatcccc acctgcccca
      301 ggaggaggtc ctgaagttgt acaggagcat gacgctgctc aacaccatgg accgcattct
      361 ctatgagtcc cagcgacagg gccggatctc cttctacatg accaactatg gcgaggaggg
      421 gacacatgtg gggagtgccg ctgccctgga gcgaacggac ctggtgtttg gccagtaccg
      481 ggaggcaggt gtgctcatgt accgggacta cccgctggag ctgttcatgg cccagtgcta
      541 cggcaacgtg agtgacccag gcaagggacg ccagatgccc gttcactacg gctgcaagga
      601 gcgtcacttc gtcaccattt cctctccact ggctacgcag atccctcagg cggtaggggc
      661 agcctatgct gccaagcggg ccaatgccaa tcagatcgtg atctgttact ttggcgaggg
      721 ggcagccagt gaaggggatg cccacgccgg tttcaatttt gctgccacct tggagtgtcc
      781 tatcatcttc ttctgccgga acaatggcta tgccatctcc acaccaacct ctgagcagta
      841 ccgtggggat ggcatagcgg ctcggggccc tgggtatggc atcatgtcaa tccgtgtgga
      901 cggcaacgat gtgtttgcgg tgtacaatgc cactaaggag gcccgacggc gggctgtggc
      961 tgagaaccag cccttcctca ttgaggccat gacctacagg atcgggcacc acagcaccag
     1021 tgacgacagc tctgcgtacc gttcggtgga tgaggtcaat tactgggaca agcaggacca
     1081 cccgatctcg agactgaggc agtacctgct gaaccagggt tggtgggatg aggaacagga
     1141 gaaggcctgg cggaagcagt cacgaaagaa ggtgatggag gcctttgaac aggctgagcg
     1201 gaagctaaag cccaacccaa gcctcctctt ctccgatgtg taccaggaga tgcctgccca
     1261 gctgcgcagg cagcaggagt ccctggctcg gcacctgcag acctacgggg agcactaccc
     1321 cctggaccac tttgacaagt gaggccctaa ccaccacaag gtggtcctgc tctgagtgga
     1381 gctggggggc ggcaggacgc tgccccccgc tgctaaccag tgctgctgac gtgctgagca
     1441 gccagtgtgg cacgcactgc ggctccccca ggctacttca ctggggcacc acatttgcag
     1501 ccattgctgc agctccatca ctcccttcac ctgttacagt gccttttagg ggtggggagg
     1561 aaccagggcc agggcagacc ctttgggcag ggtggccctg gatttgctca gagaaatagc
     1621 tgattggcag agtaagtaaa ccacagctct gcctttgaaa aaaaaaaaaa aaaaaaaaaa
     1681 aaaaaaaaaa
//
