LOCUS       NM_019267               1491 bp    mRNA    linear   ROD 09-FEB-2025
DEFINITION  Rattus norvegicus branched chain keto acid dehydrogenase E1 subunit
            beta (Bckdhb), mRNA; nuclear gene for mitochondrial product.
ACCESSION   NM_019267 XM_001062965 XM_343439
VERSION     NM_019267.1
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 1491)
  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   2  (bases 1 to 1491)
  AUTHORS   Ahmed,F., Torrado,M., Zinovieva,R.D., Senatorov,V.V., Wistow,G. and
            Tomarev,S.I.
  TITLE     Gene expression profile of the rat eye iridocorneal angle: NEIBank
            expressed sequence tag analysis
  JOURNAL   Invest Ophthalmol Vis Sci 45 (9), 3081-3090 (2004)
   PUBMED   15326124
REFERENCE   3  (bases 1 to 1491)
  AUTHORS   Kwitek,A.E., Gullings-Handley,J., Yu,J., Carlos,D.C., Orlebeke,K.,
            Nie,J., Eckert,J., Lemke,A., Andrae,J.W., Bromberg,S., Pasko,D.,
            Chen,D., Scheetz,T.E., Casavant,T.L., Soares,M.B., Sheffield,V.C.,
            Tonellato,P.J. and Jacob,H.J.
  TITLE     High-density rat radiation hybrid maps containing over 24,000
            SSLPs, genes, and ESTs provide a direct link to the rat genome
            sequence
  JOURNAL   Genome Res 14 (4), 750-757 (2004)
   PUBMED   15060019
REFERENCE   4  (bases 1 to 1491)
  AUTHORS   Doering,C.B., Coursey,C., Spangler,W. and Danner,D.J.
  TITLE     Murine branched chain alpha-ketoacid dehydrogenase kinase; cDNA
            cloning, tissue distribution, and temporal expression during
            embryonic development
  JOURNAL   Gene 212 (2), 213-219 (1998)
   PUBMED   9611264
REFERENCE   5  (bases 1 to 1491)
  AUTHORS   Lombardo,Y.B., Thamotharan,M., Bawani,S.Z., Paul,H.S. and
            Adibi,S.A.
  TITLE     Posttranscriptional alterations in protein masses of hepatic
            branched-chain keto acid dehydrogenase and its associated kinase in
            diabetes
  JOURNAL   Proc Assoc Am Physicians 110 (1), 40-49 (1998)
   PUBMED   9460082
REFERENCE   6  (bases 1 to 1491)
  AUTHORS   Zhao,Y., Kuntz,M.J., Harris,R.A. and Crabb,D.W.
  TITLE     Molecular cloning of the E1 beta subunit of the rat branched chain
            alpha-ketoacid dehydrogenase
  JOURNAL   Biochim Biophys Acta 1260 (2), 243 (1995)
   PUBMED   7841205
  REMARK    Correction to:[Biochim Biophys Acta. 1992 Sep 24;1132(2):207-10.
            doi: 10.1016/0167-4781(92)90014-q.. PMID: 1390893]
REFERENCE   7  (bases 1 to 1491)
  AUTHORS   Zhao,Y., Kuntz,M.J., Harris,R.A. and Crabb,D.W.
  TITLE     Molecular cloning of the E1 beta subunit of the rat branched chain
            alpha-ketoacid dehydrogenase
  JOURNAL   Biochim Biophys Acta 1132 (2), 207-210 (1992)
   PUBMED   1390893
  REMARK    Erratum:[Biochim Biophys Acta. 1995 Jan 25;1260(2):243.. PMID:
            7841205]
REFERENCE   8  (bases 1 to 1491)
  AUTHORS   Popov,K.M., Zhao,Y., Shimomura,Y., Kuntz,M.J. and Harris,R.A.
  TITLE     Branched-chain alpha-ketoacid dehydrogenase kinase. Molecular
            cloning, expression, and sequence similarity with histidine protein
            kinases
  JOURNAL   J Biol Chem 267 (19), 13127-13130 (1992)
   PUBMED   1377677
REFERENCE   9  (bases 1 to 1491)
  AUTHORS   Nobukuni,Y., Mitsubuchi,H., Akaboshi,I., Indo,Y., Endo,F.,
            Yoshioka,A. and Matsuda,I.
  TITLE     Maple syrup urine disease. Complete defect of the E1 beta subunit
            of the branched chain alpha-ketoacid dehydrogenase complex due to a
            deletion of an 11-bp repeat sequence which encodes a mitochondrial
            targeting leader peptide in a family with the disease
  JOURNAL   J Clin Invest 87 (5), 1862-1866 (1991)
   PUBMED   2022752
REFERENCE   10 (bases 1 to 1491)
  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
            CK473898.1, M94040.1, CB547976.1, BM385109.1 and DV215445.1.
            
            On or before Oct 28, 2007 this sequence version replaced
            XM_001062965.1, XM_343439.3.
            
            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 :: BC158861.1, FQ226240.1 [ECO:0000332]
            RNAseq introns              :: single sample supports all introns
                                           SAMD00132261, SAMD00132262
                                           [ECO:0000348]
            ##Evidence-Data-END##
            
            ##RefSeq-Attributes-START##
            gene product(s) localized to mito. :: inferred from homology
            RefSeq Select criteria             :: based on single
                                                  protein-coding transcript
            ##RefSeq-Attributes-END##
            COMPLETENESS: complete on the 3' end.
PRIMARY     REFSEQ_SPAN         PRIMARY_IDENTIFIER PRIMARY_SPAN        COMP
            1-719               CK473898.1         1-719
            720-791             M94040.1           631-702
            792-1054            CB547976.1         213-475
            1055-1336           BM385109.1         173-454             c
            1337-1491           DV215445.1         356-510
FEATURES             Location/Qualifiers
     source          1..1491
                     /organism="Rattus norvegicus"
                     /mol_type="mRNA"
                     /db_xref="taxon:10116"
                     /chromosome="8"
                     /map="8q31"
     gene            1..1491
                     /gene="Bckdhb"
                     /gene_synonym="BCKDE1B"
                     /note="branched chain keto acid dehydrogenase E1 subunit
                     beta"
                     /db_xref="GeneID:29711"
                     /db_xref="RGD:2197"
     exon            1..218
                     /gene="Bckdhb"
                     /gene_synonym="BCKDE1B"
                     /inference="alignment:Splign:2.1.0"
     CDS             29..1201
                     /gene="Bckdhb"
                     /gene_synonym="BCKDE1B"
                     /EC_number="1.2.4.4"
                     /note="2-oxoisovalerate dehydrogenase beta subunit;
                     branched chain keto acid dehydrogenase E1 beta;
                     2-oxoisovalerate dehydrogenase subunit beta,
                     mitochondrial; BCKDH E1-beta; branched-chain alpha-keto
                     acid dehydrogenase E1 component beta chain; branched chain
                     keto acid dehydrogenase E1, beta polypeptide"
                     /codon_start=1
                     /product="2-oxoisovalerate dehydrogenase subunit beta,
                     mitochondrial precursor"
                     /protein_id="NP_062140.1"
                     /db_xref="GeneID:29711"
                     /db_xref="RGD:2197"
                     /translation="MAAVAARAGGLLRLGAAGAERRRRGLRCAALVQGFLQPAVDDAS
                     QKRRVAHFTFQPDPESLQYGQTQKMNLFQSITSALDNSLAKDPTAVIFGEDVAFGGVF
                     RCTVGLRDKYGKDRVFNTPLCEQGIVGFGIGIAVTGATAIAEIQFADYIFPAFDQIVN
                     EAAKYRYRSGDLFNCGSLTIRAPWGCVGHGALYHSQSPEAFFAHCPGIKVVIPRSPFQ
                     AKGLLLSCIEDKNPCIFFEPKILYRAAVEQVPVEPYKIPLSQAEVIQEGSDVTLVAWG
                     TQVHVIREVASMAQEKLGVSCEVIDLRTIVPWDVDTVCKSVIKTGRLLISHEAPLTGG
                     FASEISSTVQEECFLNLEAPISRVCGYDTPFPHIFEPFYIPDKWKCYDALRKMINY"
     exon            219..296
                     /gene="Bckdhb"
                     /gene_synonym="BCKDE1B"
                     /inference="alignment:Splign:2.1.0"
     exon            297..365
                     /gene="Bckdhb"
                     /gene_synonym="BCKDE1B"
                     /inference="alignment:Splign:2.1.0"
     exon            366..499
                     /gene="Bckdhb"
                     /gene_synonym="BCKDE1B"
                     /inference="alignment:Splign:2.1.0"
     exon            500..655
                     /gene="Bckdhb"
                     /gene_synonym="BCKDE1B"
                     /inference="alignment:Splign:2.1.0"
     exon            656..764
                     /gene="Bckdhb"
                     /gene_synonym="BCKDE1B"
                     /inference="alignment:Splign:2.1.0"
     exon            765..862
                     /gene="Bckdhb"
                     /gene_synonym="BCKDE1B"
                     /inference="alignment:Splign:2.1.0"
     exon            863..973
                     /gene="Bckdhb"
                     /gene_synonym="BCKDE1B"
                     /inference="alignment:Splign:2.1.0"
     exon            974..1060
                     /gene="Bckdhb"
                     /gene_synonym="BCKDE1B"
                     /inference="alignment:Splign:2.1.0"
     exon            1061..1209
                     /gene="Bckdhb"
                     /gene_synonym="BCKDE1B"
                     /inference="alignment:Splign:2.1.0"
     exon            1210..1491
                     /gene="Bckdhb"
                     /gene_synonym="BCKDE1B"
                     /inference="alignment:Splign:2.1.0"
ORIGIN      
        1 cgtgggctgc ggtgcctgag aacccgggat ggcggcggtg gctgcgcgcg ccggtgggct
       61 gctgcggctc ggggctgcag gagcggagcg gcggcggcgc ggattgcgct gcgctgcgct
      121 ggtgcaaggc ttcttgcaac ccgctgtcga tgacgcgtcc cagaagcggc gggtggccca
      181 cttcactttc cagccggacc cagagtccct tcagtatggg caaactcaga aaatgaacct
      241 cttccagtca ataacaagtg ccttggataa ctcattggcc aaagacccta ctgcagtaat
      301 atttggtgag gacgttgcct ttggtggagt cttccgatgt actgttggct tacgagacaa
      361 atatggaaaa gacagagtgt ttaacacccc gttgtgtgaa caaggaattg ttgggtttgg
      421 cattggaatc gcggtcactg gtgctacagc tattgcggaa atccagtttg ccgactatat
      481 tttccctgcc tttgatcaga ttgtcaacga agctgccaag tatcgatacc gctctggcga
      541 tcttttcaac tgtgggagcc tcaccatccg ggccccttgg ggctgtgtgg gccatggggc
      601 tctttaccat tctcagagtc ctgaagcctt ttttgcccat tgcccaggaa tcaaggtggt
      661 tatacccaga agccctttcc aggccaaggg acttctgttg tcatgcatag aagataaaaa
      721 tccatgtata ttttttgaac ctaagatact ttaccgggca gcagtggaac aggtcccagt
      781 agaaccctac aagatcccct tgtctcaggc cgaagtcatc caggagggca gcgatgtgac
      841 tctggttgcc tggggcactc aggttcatgt catccgggag gtggcttcca tggcccaaga
      901 aaagcttgga gtgtcttgtg aagtcatcga tctgcggacc attgtgcctt gggatgtgga
      961 tacagtttgt aagtctgtga tcaaaacggg gcgactgttg atcagccacg aggcgccctt
     1021 gacaggcggc tttgcctctg agatcagctc cacggtccag gaagaatgtt tcttgaacct
     1081 agaggctcca atatctagag tgtgtggata tgacaccccg tttcctcaca tttttgagcc
     1141 cttttatatc ccagacaaat ggaagtgcta tgatgccctt cgcaagatga tcaactactg
     1201 acgactgaga aaaccaggaa gatcatgacc agatatggaa atatttttct gaactctttt
     1261 tatatttcct tgtacttctt tcttctcaca cgtggttttg tgagatgaac tatggcggat
     1321 actggctgac gagctgcgaa ttacttatca tggctgtgta aagtggattc ctggatctag
     1381 tgcatgaggg taacacttta gagggcacat gggcggtgat cttacgattg tttcatgact
     1441 acaaatatcc ggtggtgttg tgttcaataa agcaaaatgg catcgccctc a
//
