branched chain ketoacid dehydrogenase kinase (BCKDK) - coding DNA reference sequence

(used for variant description)

(last modified December 21, 2013)


This file was created to facilitate the description of sequence variants on transcript NM_005881.2 in the BCKDK gene based on a coding DNA reference sequence following the HGVS recommendations.

The sequence was taken from NG_033011.1, covering BCKDK transcript NM_005881.2.


Please note that introns are available by clicking on the exon numbers above the sequence.
 (upstream sequence)
                                                   .                g.5016
                                             aaactacaactcccag       c.-301

 .         .         .         .         .         .                g.5076
 gacccagcgggcgctgccgcccacgcgacgtcacggcggcggagggcgcaggcggctggg       c.-241

 .         .         .         .         .         .                g.5136
 cgcctggcgagtggactgttcgagcccttccgctgggacccgggccctggctccggcccc       c.-181

 .   | 02     .         .         .         .         .             g.5763
 gcg | atgggagctgctctccgcgggctgagcctgtcagcatcctcgacgcaccctggtccc    c.-121

 .         .         .         .         .         .                g.5823
 tgaagtcggagaagagcccctacccacccacacccccttgccccattttgggtcgcctgg       c.-61

 .         .         .         .         .         .                g.5883
 gtcctcagtcctagcggatcctcagtcctagcggccaccgggtctgaaaggagcaagacg       c.-1

          .         .         .         .         .         .       g.5943
 ATGATCCTGGCGTCGGTGCTGAGGAGCGGTCCCGGGGGCGGGCTTCCGCTCCGGCCCCTC       c.60
 M  I  L  A  S  V  L  R  S  G  P  G  G  G  L  P  L  R  P  L         p.20

          .         .         .         .         .         .       g.6003
 CTGGGACCCGCACTCGCGCTCCGGGCCCGCTCGACGTCGGCCACCGACACACACCACGTG       c.120
 L  G  P  A  L  A  L  R  A  R  S  T  S  A  T  D  T  H  H  V         p.40

          .         .         .         .         .         .       g.6063
 GAGATGGCTCGGGAGCGCTCCAAGACCGTCACCTCCTTTTACAACCAGTCGGCCATCGAC       c.180
 E  M  A  R  E  R  S  K  T  V  T  S  F  Y  N  Q  S  A  I  D         p.60

          .      | 03  .         .         .         .         .    g.6215
 GCGGCAGCGGAGAAG | CCCTCAGTCCGCCTAACGCCCACCATGATGCTCTACGCTGGCCGC    c.240
 A  A  A  E  K   | P  S  V  R  L  T  P  T  M  M  L  Y  A  G  R      p.80

          .         .     | 04   .         .         .         .    g.6368
 TCTCAGGACGGCAGCCACCTTCTG | AAAAGTGCTCGGTACCTGCAGCAAGAACTTCCAGTG    c.300
 S  Q  D  G  S  H  L  L   | K  S  A  R  Y  L  Q  Q  E  L  P  V      p.100

          .         .         .         .         .         .       g.6428
 AGGATTGCTCACCGCATCAAGGGCTTCCGCTGCCTTCCTTTCATCATTGGCTGCAACCCC       c.360
 R  I  A  H  R  I  K  G  F  R  C  L  P  F  I  I  G  C  N  P         p.120

          .      | 05  .         .         .         .         .    g.6781
 ACCATACTGCACGTG | CATGAGCTATATATCCGTGCCTTCCAGAAGCTGACAGACTTCCCT    c.420
 T  I  L  H  V   | H  E  L  Y  I  R  A  F  Q  K  L  T  D  F  P      p.140

     | 06    .         .         .         .         .         .    g.6921
 CCG | ATCAAGGACCAGGCGGACGAGGCCCAGTACTGCCAGCTGGTGCGACAGCTGCTGGAT    c.480
 P   | I  K  D  Q  A  D  E  A  Q  Y  C  Q  L  V  R  Q  L  L  D      p.160

          .         .         .         .         .         .       g.6981
 GACCACAAGGATGTGGTGACCCTCTTGGCAGAGGGCCTACGTGAGAGCCGGAAGCACATA       c.540
 D  H  K  D  V  V  T  L  L  A  E  G  L  R  E  S  R  K  H  I         p.180

     | 07    .         .         .         .         .         .    g.7117
 GAG | GATGAAAAGCTCGTCCGCTACTTCTTGGACAAGACGCTGACTTCGAGGCTTGGAATC    c.600
 E   | D  E  K  L  V  R  Y  F  L  D  K  T  L  T  S  R  L  G  I      p.200

          .         .         .         .   | 08     .         .    g.7365
 CGCATGTTGGCCACGCATCACCTGGCGCTGCATGAGGACAAG | CCTGACTTTGTCGGCATC    c.660
 R  M  L  A  T  H  H  L  A  L  H  E  D  K   | P  D  F  V  G  I      p.220

          .         .         .         .         .       | 09 .    g.7754
 ATCTGTACTCGTCTCTCACCAAAGAAGATTATTGAGAAGTGGGTGGACTTTGCCAG | ACGC    c.720
 I  C  T  R  L  S  P  K  K  I  I  E  K  W  V  D  F  A  R  |  R      p.240

          .         .         .         .         .         .       g.7814
 CTGTGTGAGCACAAGTATGGCAATGCGCCCCGTGTCCGCATCAATGGCCATGTGGCTGCC       c.780
 L  C  E  H  K  Y  G  N  A  P  R  V  R  I  N  G  H  V  A  A         p.260

          .         .         .         .         .         .       g.7874
 CGGTTCCCCTTCATCCCTATGCCACTGGACTACATCCTGCCGGAGCTGCTCAAGAATGCC       c.840
 R  F  P  F  I  P  M  P  L  D  Y  I  L  P  E  L  L  K  N  A         p.280

       | 10  .         .         .         .         .         .    g.8014
 ATGAG | AGCCACAATGGAGAGTCACCTAGACACTCCCTACAATGTCCCAGATGTGGTCATC    c.900
 M  R  |  A  T  M  E  S  H  L  D  T  P  Y  N  V  P  D  V  V  I      p.300

          .         .         .      | 11  .         .         .    g.8553
 ACCATCGCCAACAATGATGTCGATCTGATCATCAG | GATCTCAGACCGTGGTGGAGGAATC    c.960
 T  I  A  N  N  D  V  D  L  I  I  R  |  I  S  D  R  G  G  G  I      p.320

          .         .         .         .         .         .       g.8613
 GCTCACAAAGATCTGGACCGGGTCATGGACTACCACTTCACTACTGCTGAGGCCAGCACA       c.1020
 A  H  K  D  L  D  R  V  M  D  Y  H  F  T  T  A  E  A  S  T         p.340

          .         .         .         .         .         .       g.8673
 CAGGACCCCCGGATCAGCCCCCTCTTTGGCCATCTGGACATGCATAGTGGCGCCCAGTCA       c.1080
 Q  D  P  R  I  S  P  L  F  G  H  L  D  M  H  S  G  A  Q  S         p.360

          .     | 12   .         .         .         .         .    g.8826
 GGACCCATGCACGG | CTTTGGCTTCGGGTTGCCCACGTCACGGGCCTACGCGGAGTACCTC    c.1140
 G  P  M  H  G  |  F  G  F  G  L  P  T  S  R  A  Y  A  E  Y  L      p.380

          .         .         .         .         .         .       g.8886
 GGTGGGTCTCTGCAGCTGCAGTCCCTGCAGGGCATTGGCACGGACGTCTACCTGCGGCTC       c.1200
 G  G  S  L  Q  L  Q  S  L  Q  G  I  G  T  D  V  Y  L  R  L         p.400

          .         .         .                                     g.8925
 CGCCACATCGATGGCCGGGAGGAAAGCTTCCGGATCTGA                            c.1239
 R  H  I  D  G  R  E  E  S  F  R  I  X                              p.412

          .         .         .         .         .         .       g.8985
 ccccacagcctttggcctgctcacccgaccagcctgggccgcattccctgcaggacctcc       c.*60

          .         .         .         .         .         .       g.9045
 cgggtcaggcagggcggccccctgctccacacactgctgcatcttgggtctcagggaccc       c.*120

          .         .         .         .         .         .       g.9105
 agacagatggacttacatggagctgggcactgccctgcctcaacagggtccattgcctcc       c.*180

          .         .         .         .         .         .       g.9165
 tcgcctccagaacttggagcagggaagtgggcaccctgaggcctccagcaccagttccgt       c.*240

          .         .         .         .         .         .       g.9225
 cattctcgttcctggggaacccccactctgacctgttattaaagttcacattttgaatgc       c.*300

          .         .         .         .         .         .       g.9285
 cctctcgggccccgtgtgtggggagggcaggtgaacttttgtttctgcccccattcaggt       c.*360

          .         .         .         .         .         .       g.9345
 tcactgagcccttgggttgaactggttcgtgtcccagtctcttacctgccctgagagcct       c.*420

          .         .         .         .         .         .       g.9405
 ggcaggccaggagtagaatgggtcccaagtctgttgcatgtttgatttggtgggagtggg       c.*480

          .         .         .         .                           g.9451
 atgactgcagcaccttatacaaagagctttcattcatcttgttgaa                     c.*526

 (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 ketoacid dehydrogenase kinase 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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