branched chain keto acid dehydrogenase E1, beta polypeptide (BCKDHB) - coding DNA reference sequence

(used for variant description)

(last modified May 25, 2024)


This file was created to facilitate the description of sequence variants on transcript NM_183050.2 in the BCKDHB gene based on a coding DNA reference sequence following the HGVS recommendations.

The sequence was taken from NC_000006.11, covering BCKDHB transcript NM_183050.2.


Please note that introns are available by clicking on the exon numbers above the sequence.
 (upstream sequence)
                                                                    g.5007
                                                      gggtgct       c.-61

 .         .         .         .         .         .                g.5067
 ccgccctccccgcaggcggcgtgcggctgcatagcctgagaatcccggtggtgagcgggg       c.-1

          .         .         .         .         .         .       g.5127
 ATGGCGGTTGTAGCGGCGGCTGCCGGCTGGCTACTCAGGCTCAGGGCGGCAGGGGCTGAG       c.60
 M  A  V  V  A  A  A  A  G  W  L  L  R  L  R  A  A  G  A  E         p.20

          .         .         .         .         .         .       g.5187
 GGGCACTGGCGTCGGCTTCCTGGCGCGGGGCTGGCGCGGGGCTTTTTGCACCCCGCCGCG       c.120
 G  H  W  R  R  L  P  G  A  G  L  A  R  G  F  L  H  P  A  A         p.40

          .         .         .         .         .         .       g.5247
 ACTGTCGAGGATGCGGCCCAGAGGCGGCAGGTGGCTCATTTTACTTTCCAGCCAGATCCG       c.180
 T  V  E  D  A  A  Q  R  R  Q  V  A  H  F  T  F  Q  P  D  P         p.60

          .       | 02 .         .         .         .         .    g.25964
 GAGCCCCGGGAGTACG | GGCAAACTCAGAAAATGAATCTTTTCCAGTCTGTAACAAGTGCC    c.240
 E  P  R  E  Y  G |   Q  T  Q  K  M  N  L  F  Q  S  V  T  S  A      p.80

          .         .         .     | 03   .         .         .    g.27560
 TTGGATAACTCATTGGCCAAAGATCCTACTGCAG | TAATATTTGGTGAAGATGTTGCCTTT    c.300
 L  D  N  S  L  A  K  D  P  T  A  V |   I  F  G  E  D  V  A  F      p.100

          .         .         .         .    | 04    .         .    g.66068
 GGTGGAGTCTTTAGATGCACTGTTGGCTTGCGAGACAAATATG | GAAAAGATAGAGTTTTT    c.360
 G  G  V  F  R  C  T  V  G  L  R  D  K  Y  G |   K  D  R  V  F      p.120

          .         .         .         .         .         .       g.66128
 AATACCCCATTGTGTGAACAAGGAATTGTTGGATTTGGAATCGGAATTGCGGTCACTGGA       c.420
 N  T  P  L  C  E  Q  G  I  V  G  F  G  I  G  I  A  V  T  G         p.140

          .         .         .         .         .        | 05.    g.67251
 GCTACTGCCATTGCGGAAATTCAGTTTGCAGATTATATTTTCCCTGCATTTGATCAG | ATT    c.480
 A  T  A  I  A  E  I  Q  F  A  D  Y  I  F  P  A  F  D  Q   | I      p.160

          .         .         .         .         .         .       g.67311
 GTTAATGAAGCTGCCAAGTATCGCTATCGCTCTGGGGATCTTTTTAACTGTGGAAGCCTC       c.540
 V  N  E  A  A  K  Y  R  Y  R  S  G  D  L  F  N  C  G  S  L         p.180

          .         .         .         .         .         .       g.67371
 ACTATCCGGTCCCCTTGGGGCTGTGTTGGTCATGGGGCTCTCTATCATTCTCAGAGTCCT       c.600
 T  I  R  S  P  W  G  C  V  G  H  G  A  L  Y  H  S  Q  S  P         p.200

          .         .         .    | 06    .         .         .    g.69682
 GAAGCATTTTTTGCCCATTGCCCAGGAATCAAG | GTGGTTATACCCAGAAGCCCTTTCCAG    c.660
 E  A  F  F  A  H  C  P  G  I  K   | V  V  I  P  R  S  P  F  Q      p.220

          .         .         .         .         .         .       g.69742
 GCCAAAGGACTTCTTTTGTCATGCATAGAGGATAAAAATCCTTGTATATTTTTTGAACCT       c.720
 A  K  G  L  L  L  S  C  I  E  D  K  N  P  C  I  F  F  E  P         p.240

          .         .   | 07     .         .         .         .    g.99345
 AAAATACTTTACAGGGCAGCAG | CGGAAGAAGTCCCTATAGAACCATACAACATCCCACTG    c.780
 K  I  L  Y  R  A  A  A |   E  E  V  P  I  E  P  Y  N  I  P  L      p.260

          .         .         .         .         .         .       g.99405
 TCCCAGGCCGAAGTCATACAGGAAGGGAGTGATGTTACTCTAGTTGCCTGGGGCACTCAG       c.840
 S  Q  A  E  V  I  Q  E  G  S  D  V  T  L  V  A  W  G  T  Q         p.280

  | 08       .         .         .         .         .         .    g.101535
  | GTTCATGTGATCCGAGAGGTAGCTTCCATGGCAAAAGAAAAGCTTGGAGTGTCTTGTGAA    c.900
  | V  H  V  I  R  E  V  A  S  M  A  K  E  K  L  G  V  S  C  E      p.300

          .         .         .         .         .  | 09      .    g.171517
 GTCATTGATCTGAGGACTATAATACCTTGGGATGTGGACACAATTTGTAAG | TCTGTGATC    c.960
 V  I  D  L  R  T  I  I  P  W  D  V  D  T  I  C  K   | S  V  I      p.320

          .         .         .         .         .         .       g.171577
 AAAACAGGGCGACTGCTAATCAGTCACGAGGCTCCCTTGACAGGCGGCTTTGCATCGGAA       c.1020
 K  T  G  R  L  L  I  S  H  E  A  P  L  T  G  G  F  A  S  E         p.340

          .         | 10         .         .         .         .    g.242079
 ATCAGCTCTACAGTTCAG | GAGGAATGTTTCTTGAACCTAGAGGCTCCTATATCAAGAGTA    c.1080
 I  S  S  T  V  Q   | E  E  C  F  L  N  L  E  A  P  I  S  R  V      p.360

          .         .         .         .         .         .       g.242139
 TGTGGTTATGACACACCATTTCCTCACATTTTTGAACCATTCTACATCCCAGACAAATGG       c.1140
 C  G  Y  D  T  P  F  P  H  I  F  E  P  F  Y  I  P  D  K  W         p.380

          .         .         .                                     g.242178
 AAGTGTTATGATGCCCTTCGAAAAATGATCAACTATTGA                            c.1179
 K  C  Y  D  A  L  R  K  M  I  N  Y  X                              p.392

          .         .         .         .         .         .       g.242238
 ccatataggtaggtatgcatcttgagaaagctactatgtgcccctgacattaacgtactg       c.*60

          .         .         .         .         .         .       g.242298
 ttaaccaagacacagcaatcatcagtgttttgatggtaacaaactttgatggtaaagttg       c.*120

          .         .         .         .         .         .       g.242358
 gtaaaaggcaactttcagaagaaaataatgtgctttagaaaaaaaattcaaatttatagt       c.*180

          .         .         .         .         .         .       g.242418
 agtatatttacatttttgttgttgttgttgttctgagatggagtctcactctgtcgccca       c.*240

          .         .         .         .         .         .       g.242478
 ggctagactgcagtggtgcaatctcagctcactgcaacctcccccctacccctgagttca       c.*300

          .         .         .         .         .         .       g.242538
 agcgattctcctgcctcagcctgctgagtagttgggattacaggcgcccaccaccatgcc       c.*360

          .         .         .         .         .         .       g.242598
 cagctaatttttgtgtttttattggaggtggggtgtcactatgttggctcagctgatttc       c.*420

          .         .         .         .         .         .       g.242658
 aaattcctgacctcaagtgatccacctgccttagcctcccaaagtgctgggattacaggc       c.*480

          .         .         .         .         .         .       g.242718
 atgagccactgcacctggctatatttacatttaatagaaacatatctagcatatgtatat       c.*540

          .         .         .         .         .         .       g.242778
 gtgattttttttttttttttgagaccgagtctcactctgtcaccaggctggagtgcagtg       c.*600

          .         .         .         .         .         .       g.242838
 gtgcgatcttggctcatggcaacctctgcctcccaggttcaagcgattgtcctgcctcag       c.*660

          .         .         .         .         .         .       g.242898
 tctcctgagtagctgggactacaggtgtgcaccaacatgcccagctaattttttatattt       c.*720

          .         .         .         .         .         .       g.242958
 ttagtagagacggggtttcaccatgttggccaggatggtctcgatctcttgaccttgtgt       c.*780

          .         .         .         .         .         .       g.243018
 tccacccgccttggcctcccaaagtgctggaattacaggcatgagccaccgcgcctggcc       c.*840

          .         .         .         .         .         .       g.243078
 tgtatatgtgatttctaaaaaatagatgcatgcatatgttaacattgaatagtcaatcac       c.*900

          .         .         .         .         .         .       g.243138
 tagatgaagatgctctctaccatggtttagattgcaagtgtactttataccattgtttca       c.*960

          .         .         .         .         .         .       g.243198
 acttcaacctttatttttgtatatattttttcagctacttaaactgttcatgaataggcg       c.*1020

          .         .         .         .         .         .       g.243258
 tactttctgtatttaaaaatggcccctcaagcaccgttaatttacattccagttatttac       c.*1080

          .         .         .         .         .         .       g.243318
 atgataattcatgacattctgaaacttgcctgtatattatctgaaaaatggatttcttga       c.*1140

          .         .         .         .         .         .       g.243378
 gcaaaagatctgtttattgtatgtaaggaaaaattttacctgaaaacaaacaaacaaacc       c.*1200

          .         .         .         .         .         .       g.243438
 ctaaaactcagcaccactaccatttccagaagcttttttaacaagtgaatattttttaca       c.*1260

          .         .         .         .         .         .       g.243498
 taatggataataaatggtatttattcatcatgattttctaagtaatgcttatcagcccct       c.*1320

          .         .         .         .         .         .       g.243558
 tcagtggtgttattctaacaaaatgaatacattacaaattattaagttagcttttcagag       c.*1380

          .         .         .         .         .         .       g.243618
 ttttaatatagaagttactagcactgacacactgattttgaaatgtttcaaaatgagtag       c.*1440

          .         .         .         .         .         .       g.243678
 atatgattagatcctttaccttttaatatctagtttttccaaaaatgatagtaatatctt       c.*1500

          .         .         .         .         .         .       g.243738
 ttgaagaactatgctttttcaaaagcaaaatcaactctgtataatagcacattctctact       c.*1560

          .         .         .         .         .         .       g.243798
 ttttaaagatcagaaatgctagaattcttgacttttgtgtatgggtagtaaatctagctc       c.*1620

          .         .         .         .         .         .       g.243858
 actgaaaatcagagtgaaacgcctttacatttgtgcggatagagaagttaactctccctc       c.*1680

          .         .         .         .         .         .       g.243918
 atatgtcactgtgctaccataatccctacattttctgtctgttctagtctaagaatattg       c.*1740

          .         .         .         .         .         .       g.243978
 ttatagatggaagttaggaccattagcccacagatgcatgtattctctcagacataccgg       c.*1800

          .         .         .         .         .         .       g.244038
 tgtagatgctatatttctgatgtcttcttaatgtctgtgaaagcaactggcatctacaat       c.*1860

          .         .         .         .         .         .       g.244098
 aaatcacacttagagctggttagaggactccttcacttttgttgtccatgtggttccctt       c.*1920

          .         .         .         .         .         .       g.244158
 ccgtggaccagccgtaataaagagccaaggtagtgatggtggccacgtgcctcgttgcta       c.*1980

          .         .         .         .         .         .       g.244218
 tttttaaagtaatattcagatgtggttttaaatttagattatgtattccttttgaaacat       c.*2040

          .         .         .         .         .         .       g.244278
 aagaaaaacatttaaacctatgctgaaaatacgataaaagaaaaacaactccaatatgct       c.*2100

          .         .         .         .         .         .       g.244338
 aaaagttaaatatggtatttaagaaaatagtcatgtatgcaattgagaaagtctataatt       c.*2160

          .         .         .         .         .         .       g.244398
 tattttacagaaaagctggaagattatgactagatatggaaatattttttctgaattttt       c.*2220

          .         .         .         .         .         .       g.244458
 ttttatatttcctccgacttacctctttttgaaaagagagtttttattaagtgaaccatc       c.*2280

          .         .         .         .         .         .       g.244518
 acgatattggctgaaaagttctacattctattattgtattgtaacacacatgtattgatg       c.*2340

          .         .         .         .         .         .       g.244578
 attttcattaagagtttcagattaactttgaaaaatattccacatggtaatcttataaat       c.*2400

          .         .         .         .         .         .       g.244638
 tctgtttaattacatctgtaaatattatgtgtgtgatagtattcaataaagtaaaatcaa       c.*2460

                                                                    g.244644
 attgtc                                                             c.*2466

 (downstream sequence)
Legend:
Nucleotide numbering (following the rules of the HGVS for a 'Coding DNA Reference Sequence') is indicated at the right of the sequence, counting the A of the ATG translation initiating Methionine as 1. Every 10th nucleotide is indicated by a "." above the sequence. The Branched chain keto acid dehydrogenase E1, beta polypeptide protein sequence is shown below the coding DNA sequence, with numbering indicated at the right starting with 1 for the translation initiating Methionine. Every 10th amino acid is shown in bold. The position of introns is indicated by a vertical line, splitting the two exons. The start of the first exon (transcription initiation site) is indicated by a '\', the end of the last exon (poly-A addition site) by a '/'. The exon number is indicated above the first nucleotide(s) of the exon. To aid the description of frame shift variants, all stop codons in the +1 frame are shown in bold while all stop codons in the +2 frame are underlined.

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