phenylalanine hydroxylase (PAH) - coding DNA reference sequence

(used for variant description)

(last modified October 7, 2013)


This file was created to facilitate the description of sequence variants on transcript NM_000277.1 in the PAH gene based on a coding DNA reference sequence following the HGVS recommendations.

The sequence was taken from NG_008690.1, covering PAH transcript NM_000277.1.


Please note that introns are available by clicking on the exon numbers above the sequence.
 (upstream sequence)
           .         .         .         .         .                g.5053
        cagctgggggtaaggggggcggattattcatataattgttataccagacggtc       c.-421

 .         .         .         .         .         .                g.5113
 gcaggcttagtccaattgcagagaactcgcttcccaggcttctgagagtcccggaagtgc       c.-361

 .         .         .         .         .         .                g.5173
 ctaaacctgtctaatcgacggggcttgggtggcccgtcgctccctggcttcttcccttta       c.-301

 .         .         .         .         .         .                g.5233
 cccagggcgggcagcgaagtggtgcctcctgcgtcccccacaccctccctcagcccctcc       c.-241

 .         .         .         .         .         .                g.5293
 cctccggcccgtcctgggcaggtgacctggagcatccggcaggctgccctggcctcctgc       c.-181

 .         .         .         .         .         .                g.5353
 gtcaggacaacgcccacgaggggcgttactgtgcggagatgcaccacgcaagagacaccc       c.-121

 .         .         .         .         .         .                g.5413
 tttgtaactctcttctcctccctagtgcgaggttaaaaccttcagccccacgtgctgttt       c.-61

 .         .         .         .         .         .                g.5473
 gcaaacctgcctgtacctgaggccctaaaaagccagagacctcactcccggggagccagc       c.-1

          .         .         .         .         .         .       g.5533
 ATGTCCACTGCGGTCCTGGAAAACCCAGGCTTGGGCAGGAAACTCTCTGACTTTGGACAG       c.60
 M  S  T  A  V  L  E  N  P  G  L  G  R  K  L  S  D  F  G  Q         p.20

  | 02       .         .         .         .         .         .    g.9765
  | GAAACAAGCTATATTGAAGACAACTGCAATCAAAATGGTGCCATATCACTGATCTTCTCA    c.120
  | E  T  S  Y  I  E  D  N  C  N  Q  N  G  A  I  S  L  I  F  S      p.40

          .         .         .         .         | 03         .    g.27697
 CTCAAAGAAGAAGTTGGTGCATTGGCCAAAGTATTGCGCTTATTTGAG | GAGAATGATGTA    c.180
 L  K  E  E  V  G  A  L  A  K  V  L  R  L  F  E   | E  N  D  V      p.60

          .         .         .         .         .         .       g.27757
 AACCTGACCCACATTGAATCTAGACCTTCTCGTTTAAAGAAAGATGAGTATGAATTTTTC       c.240
 N  L  T  H  I  E  S  R  P  S  R  L  K  K  D  E  Y  E  F  F         p.80

          .         .         .         .         .         .       g.27817
 ACCCATTTGGATAAACGTAGCCTGCCTGCTCTGACAAACATCATCAAGATCTTGAGGCAT       c.300
 T  H  L  D  K  R  S  L  P  A  L  T  N  I  I  K  I  L  R  H         p.100

          .         .         .         .         .   | 04     .    g.45061
 GACATTGGTGCCACTGTCCATGAGCTTTCACGAGATAAGAAGAAAGACACAG | TGCCCTGG    c.360
 D  I  G  A  T  V  H  E  L  S  R  D  K  K  K  D  T  V |   P  W      p.120

          .         .         .         .         .         .       g.45121
 TTCCCAAGAACCATTCAAGAGCTGGACAGATTTGCCAATCAGATTCTCAGCTATGGAGCG       c.420
 F  P  R  T  I  Q  E  L  D  R  F  A  N  Q  I  L  S  Y  G  A         p.140

          .         .  | 05      .         .         .         .    g.55979
 GAACTGGATGCTGACCACCCT | GGTTTTAAAGATCCTGTGTACCGTGCAAGACGGAAGCAG    c.480
 E  L  D  A  D  H  P   | G  F  K  D  P  V  Y  R  A  R  R  K  Q      p.160

          .         .          | 06        .         .         .    g.67302
 TTTGCTGACATTGCCTACAACTACCGCCA | TGGGCAGCCCATCCCTCGAGTGGAATACATG    c.540
 F  A  D  I  A  Y  N  Y  R  H  |  G  Q  P  I  P  R  V  E  Y  M      p.180

          .         .         .         .         .         .       g.67362
 GAGGAAGAAAAGAAAACATGGGGCACAGTGTTCAAGACTCTGAAGTCCTTGTATAAAACC       c.600
 E  E  E  K  K  T  W  G  T  V  F  K  T  L  K  S  L  Y  K  T         p.200

          .         .         .         .         .         .       g.67422
 CATGCTTGCTATGAGTACAATCACATTTTTCCACTTCTTGAAAAGTACTGTGGCTTCCAT       c.660
 H  A  C  Y  E  Y  N  H  I  F  P  L  L  E  K  Y  C  G  F  H         p.220

          .         .         .         .       | 07 .         .    g.69667
 GAAGATAACATTCCCCAGCTGGAAGACGTTTCTCAGTTCCTGCAGA | CTTGCACTGGTTTC    c.720
 E  D  N  I  P  Q  L  E  D  V  S  Q  F  L  Q  T |   C  T  G  F      p.240

          .         .         .         .         .         .       g.69727
 CGCCTCCGACCTGTGGCTGGCCTGCTTTCCTCTCGGGATTTCTTGGGTGGCCTGGCCTTC       c.780
 R  L  R  P  V  A  G  L  L  S  S  R  D  F  L  G  G  L  A  F         p.260

          .         .         .         .         .         .       g.69787
 CGAGTCTTCCACTGCACACAGTACATCAGACATGGATCCAAGCCCATGTATACCCCCGAA       c.840
 R  V  F  H  C  T  Q  Y  I  R  H  G  S  K  P  M  Y  T  P  E         p.280

    | 08     .         .         .         .         .         .    g.70905
 CC | TGACATCTGCCATGAGCTGTTGGGACATGTGCCCTTGTTTTCAGATCGCAGCTTTGCC    c.900
 P  |  D  I  C  H  E  L  L  G  H  V  P  L  F  S  D  R  S  F  A      p.300

          .   | 09     .         .         .         .         .    g.75700
 CAGTTTTCCCAG | GAAATTGGCCTTGCCTCTCTGGGTGCACCTGATGAATACATTGAAAAG    c.960
 Q  F  S  Q   | E  I  G  L  A  S  L  G  A  P  D  E  Y  I  E  K      p.320

           | 10        .         .         .         .         .    g.78223
 CTCGCCACA | ATTTACTGGTTTACTGTGGAGTTTGGGCTCTGCAAACAAGGAGACTCCATA    c.1020
 L  A  T   | I  Y  W  F  T  V  E  F  G  L  C  K  Q  G  D  S  I      p.340

          .         .         .         .      | 11  .         .    g.78839
 AAGGCATATGGTGCTGGGCTCCTGTCATCCTTTGGTGAATTACAG | TACTGCTTATCAGAG    c.1080
 K  A  Y  G  A  G  L  L  S  S  F  G  E  L  Q   | Y  C  L  S  E      p.360

          .         .         .         .         .         .       g.78899
 AAGCCAAAGCTTCTCCCCCTGGAGCTGGAGAAGACAGCCATCCAAAATTACACTGTCACG       c.1140
 K  P  K  L  L  P  L  E  L  E  K  T  A  I  Q  N  Y  T  V  T         p.380

          .         .         .         .         .          | 12    g.82089
 GAGTTCCAGCCCCTCTATTACGTGGCAGAGAGTTTTAATGATGCCAAGGAGAAAGTAAG | G    c.1200
 E  F  Q  P  L  Y  Y  V  A  E  S  F  N  D  A  K  E  K  V  R  |      p.400

          .         .         .         .         .         .       g.82149
 AACTTTGCTGCCACAATACCTCGGCCCTTCTCAGTTCGCTACGACCCATACACCCAAAGG       c.1260
 N  F  A  A  T  I  P  R  P  F  S  V  R  Y  D  P  Y  T  Q  R         p.420

          .         .         .         .         .      | 13  .    g.83390
 ATTGAGGTCTTGGACAATACCCAGCAGCTTAAGATTTTGGCTGATTCCATTAACA | GTGAA    c.1320
 I  E  V  L  D  N  T  Q  Q  L  K  I  L  A  D  S  I  N  S |   E      p.440

          .         .         .                                     g.83429
 ATTGGAATCCTTTGCAGTGCCCTCCAGAAAATAAAGTAA                            c.1359
 I  G  I  L  C  S  A  L  Q  K  I  K  X                              p.452

          .         .         .         .         .         .       g.83489
 agccatggacagaatgtggtctgtcagctgtgaatctgttgatggagatccaactatttc       c.*60

          .         .         .         .         .         .       g.83549
 tttcatcagaaaaagtccgaaaagcaaaccttaatttgaaataacagccttaaatccttt       c.*120

          .         .         .         .         .         .       g.83609
 acaagatggagaaacaacaaataagtcaaaataatctgaaatgacaggatatgagtacat       c.*180

          .         .         .         .         .         .       g.83669
 actcaagagcataatggtaaatcttttggggtcatctttgatttagagatgataatccca       c.*240

          .         .         .         .         .         .       g.83729
 tactctcaattgagttaaatcagtaatctgtcgcatttcatcaagattaattaaaatttg       c.*300

          .         .         .         .         .         .       g.83789
 ggacctgcttcattcaagcttcatatatgctttgcagagaactcataaaggagcatataa       c.*360

          .         .         .         .         .         .       g.83849
 ggctaaatgtaaaacacaagactgtcattagaattgaattattgggcttaatataaatcg       c.*420

          .         .         .         .         .         .       g.83909
 taacctatgaagtttattttctattttagttaactatgattccaattactactttgttat       c.*480

          .         .         .         .         .         .       g.83969
 tgtacctaagtaaattttctttaagtcagaagcccattaaaatagttacaagcattgaac       c.*540

          .         .         .         .         .         .       g.84029
 ttctttagtattatattaatataaaaacatttttgtatgttttattgtaatcataaatac       c.*600

          .         .         .         .         .         .       g.84089
 tgctgtataaggtaataaaactctgcacctaatccccataacttccagtatcattttcca       c.*660

          .         .         .         .         .         .       g.84149
 attaattatcaagtctgttttgggaaacactttgaggacatttatgatgcagcagatgtt       c.*720

          .         .         .         .         .         .       g.84209
 gactaaaggcttggttggtagatattcaggaaatgttcactgaataaataagtaaataca       c.*780

          .         .         .         .         .         .       g.84269
 ttattgaaaagcaaatctgtataaatgtgaaatttttatttgtattagtaataaaacatt       c.*840

                                                                    g.84278
 agtagttta                                                          c.*849

 (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 Phenylalanine hydroxylase 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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