nephronophthisis 1 (juvenile) (NPHP1) - coding DNA reference sequence

(used for variant description)

(last modified May 29, 2020)


This file was created to facilitate the description of sequence variants on transcript NM_000272.3 in the NPHP1 gene based on a coding DNA reference sequence following the HGVS recommendations.

The sequence was taken from NG_008287.1, covering NPHP1 transcript NM_000272.3.


Please note that introns are available by clicking on the exon numbers above the sequence.
 (upstream sequence)
                               .         .         .                g.5034
                           ctgggaggcgggcgcacatcgatggcgtcacctt       c.-61

 .         .         .         .         .         .                g.5094
 ctggcgccgccggttggtttccctggcaactggagcaatcagagcaccgcagccagggag       c.-1

          .         .         .         .         .         .       g.5154
 ATGCTGGCGAGACGACAGCGAGATCCTCTCCAGGCCCTGCGGCGCCGCAATCAGGAGCTG       c.60
 M  L  A  R  R  Q  R  D  P  L  Q  A  L  R  R  R  N  Q  E  L         p.20

           | 02        .         .         .         .         .    g.8619
 AAGCAACAG | GTTGATAGTTTGCTTTCTGAGAGCCAACTGAAAGAAGCTCTAGAACCCAAT    c.120
 K  Q  Q   | V  D  S  L  L  S  E  S  Q  L  K  E  A  L  E  P  N      p.40

          .         .    | 03    .         .         .         .    g.30415
 AAAAGACAACATATTTATCAAAG | ATGTATCCAGTTAAAGCAGGCAATAGATGAAAATAAA    c.180
 K  R  Q  H  I  Y  Q  R  |  C  I  Q  L  K  Q  A  I  D  E  N  K      p.60

          .         .     | 04   .         .         .         .    g.31551
 AATGCTCTTCAAAAATTAAGCAAA | GCTGATGAATCTGCACCTGTTGCAAACTATAATCAG    c.240
 N  A  L  Q  K  L  S  K   | A  D  E  S  A  P  V  A  N  Y  N  Q      p.80

          .         .         .         .         .         .       g.31611
 AGAAAAGAAGAGGAGCATACTCTTTTGGACAAGCTTACCCAACAACTGCAGGGCCTTGCT       c.300
 R  K  E  E  E  H  T  L  L  D  K  L  T  Q  Q  L  Q  G  L  A         p.100

          .         .          | 05        .         .         .    g.40095
 GTGACAATAAGCAGAGAAAATATAACTGA | AGTTGGGGCACCTACTGAAGAAGAGGAAGAA    c.360
 V  T  I  S  R  E  N  I  T  E  |  V  G  A  P  T  E  E  E  E  E      p.120

          .         .         .         .         .         .       g.40155
 AGTGAAAGTGAAGATAGTGAAGACAGTGGTGGGGAGGAAGAAGATGCAGAGGAGGAAGAG       c.420
 S  E  S  E  D  S  E  D  S  G  G  E  E  E  D  A  E  E  E  E         p.140

          .         .         .         .         .         .       g.40215
 GAAGAGAAAGAGGAAAATGAATCTCACAAATGGTCAACCGGTGAAGAATACATCGCTGTT       c.480
 E  E  K  E  E  N  E  S  H  K  W  S  T  G  E  E  Y  I  A  V         p.160

          .         .         .         .   | 06     .         .    g.41527
 GGAGATTTTACTGCTCAGCAAGTTGGAGATCTTACATTTAAG | AAAGGGGAAATTCTCCTT    c.540
 G  D  F  T  A  Q  Q  V  G  D  L  T  F  K   | K  G  E  I  L  L      p.180

          .         .         .         .         .         .       g.41587
 GTAATTGAAAAAAAACCTGATGGTTGGTGGATAGCTAAGGATGCCAAAGGAAATGAAGGT       c.600
 V  I  E  K  K  P  D  G  W  W  I  A  K  D  A  K  G  N  E  G         p.200

          .         .     | 07   .         .         .         .    g.44943
 CTTGTTCCCAGAACCTACCTAGAG | CCTTATAGTGAAGAAGAAGAAGGCCAAGAGTCAAGT    c.660
 L  V  P  R  T  Y  L  E   | P  Y  S  E  E  E  E  G  Q  E  S  S      p.220

          .         .         .         .         .         .       g.45003
 GAAGAGGGCAGTGAAGAAGATGTAGAGGCGGTGGATGAAACAGCAGATGGAGCAGAAGTT       c.720
 E  E  G  S  E  E  D  V  E  A  V  D  E  T  A  D  G  A  E  V         p.240

          | 08         .         .         .         .         .    g.45384
 AAGCAAAG | AACTGATCCCCACTGGAGTGCTGTTCAGAAAGCGATTTCAGAGGCGGGCATC    c.780
 K  Q  R  |  T  D  P  H  W  S  A  V  Q  K  A  I  S  E  A  G  I      p.260

          .         .         .         .         .         .       g.45444
 TTCTGTCTTGTTAATCATGTCTCGTTTTGCTACCTAATAGTTCTGATGCGAAATAGGATG       c.840
 F  C  L  V  N  H  V  S  F  C  Y  L  I  V  L  M  R  N  R  M         p.280

          .         .         .         .         .         .       g.45504
 GAGACTGTGGAAGACACCAATGGATCTGAAACAGGGTTCAGGGCATGGAATGTACAGAGC       c.900
 E  T  V  E  D  T  N  G  S  E  T  G  F  R  A  W  N  V  Q  S         p.300

          .         .         .          | 09        .         .    g.46948
 AGAGGACGTATATTTCTGGTTTCTAAGCCTGTGCTCCAA | CAGATAAACACTGTTGATGTG    c.960
 R  G  R  I  F  L  V  S  K  P  V  L  Q   | Q  I  N  T  V  D  V      p.320

          .         .         .         .         .         .       g.47008
 TTAACTACGATGGGAGCTATTCCTGCAGGGTTCAGGCCTTCCACGCTCTCACAGCTTCTG       c.1020
 L  T  T  M  G  A  I  P  A  G  F  R  P  S  T  L  S  Q  L  L         p.340

         | 10.         .         .         .         .         .    g.48418
 GAGGAAG | GGAATCAATTTCGAGCAAATTACTTCTTACAACCAGAGCTCATGCCTTCACAA    c.1080
 E  E  G |   N  Q  F  R  A  N  Y  F  L  Q  P  E  L  M  P  S  Q      p.360

          .         .         .         .   | 11     .         .    g.49825
 CTGGCCTTCAGAGATCTGATGTGGGATGCTACAGAAGGCACT | ATTAGGTCGAGACCAAGT    c.1140
 L  A  F  R  D  L  M  W  D  A  T  E  G  T   | I  R  S  R  P  S      p.380

          .         .         .         .         .         .       g.49885
 CGTATTTCATTGATTCTGACATTATGGAGCTGTAAAATGATTCCTCTTCCAGGAATGAGC       c.1200
 R  I  S  L  I  L  T  L  W  S  C  K  M  I  P  L  P  G  M  S         p.400

          .         .         .         .         .  | 12      .    g.59815
 ATACAGGTTCTCAGCAGACATGTACGCCTCTGTCTATTTGATGGTAATAAG | GTTCTGAGC    c.1260
 I  Q  V  L  S  R  H  V  R  L  C  L  F  D  G  N  K   | V  L  S      p.420

          .         .         .         .         .         .       g.59875
 AACATTCATACAGTCAGAGCCACATGGCAACCTAAAAAGCCCAAAACATGGACCTTTTCT       c.1320
 N  I  H  T  V  R  A  T  W  Q  P  K  K  P  K  T  W  T  F  S         p.440

        | 13 .         .         .         .         .         .    g.62090
 CCCCAG | GTTACTCGCATCTTACCATGTTTGCTTGATGGTGATTGCTTTATCAGGTCTAAT    c.1380
 P  Q   | V  T  R  I  L  P  C  L  L  D  G  D  C  F  I  R  S  N      p.460

          .         .         .         .         .        | 14.    g.63230
 TCTGCATCTCCAGATCTTGGAATATTATTTGAACTTGGAATTTCTTATATTCGCAAT | TCA    c.1440
 S  A  S  P  D  L  G  I  L  F  E  L  G  I  S  Y  I  R  N   | S      p.480

          .         .         .         .         .         .       g.63290
 ACTGGTGAAAGAGGAGAGTTAAGCTGTGGCTGGGTGTTTCTTAAACTTTTTGATGCCAGT       c.1500
 T  G  E  R  G  E  L  S  C  G  W  V  F  L  K  L  F  D  A  S         p.500

          .         . | 15       .         .         .         .    g.65533
 GGAGTTCCTATTCCAGCAAA | AACTTATGAGCTTTTCTTGAATGGTGGTACTCCTTATGAA    c.1560
 G  V  P  I  P  A  K  |  T  Y  E  L  F  L  N  G  G  T  P  Y  E      p.520

          .         .         .        | 16.         .         .    g.66444
 AAAGGTATTGAAGTGGACCCTTCAATATCCAGAAGAG | CACACGGCAGTGTTTTCTACCAG    c.1620
 K  G  I  E  V  D  P  S  I  S  R  R  A |   H  G  S  V  F  Y  Q      p.540

          .         .         .         .         .         .       g.66504
 ATTATGACAATGAGAAGGCAGCCTCAACTTCTAGTGAAACTGAGATCCTTGAACAGAAGA       c.1680
 I  M  T  M  R  R  Q  P  Q  L  L  V  K  L  R  S  L  N  R  R         p.560

          .        | 17.         .         .         .         .    g.78314
 TCAAGAAATGTACTAAG | TCTACTGCCAGAAACATTAATTGGAAATATGTGTTCTATTCAC    c.1740
 S  R  N  V  L  S  |  L  L  P  E  T  L  I  G  N  M  C  S  I  H      p.580

          .         .         .         .         .         .       g.78374
 TTGTTGATATTTTATCGACAAATTCTTGGAGATGTGCTCCTGAAAGACAGGATGAGCTTG       c.1800
 L  L  I  F  Y  R  Q  I  L  G  D  V  L  L  K  D  R  M  S  L         p.600

          . | 18       .         .         .         .         .    g.80853
 CAAAGTACTG | ATTTAATTAGCCATCCCATGCTGGCCACCTTCCCCATGCTCTTGGAGCAG    c.1860
 Q  S  T  D |   L  I  S  H  P  M  L  A  T  F  P  M  L  L  E  Q      p.620

          .         .     | 19   .         .         .         .    g.84417
 CCTGATGTGATGGATGCTCTCAGG | AGTTCGTGGGCTGGAAAAGAAAGCACATTAAAAAGA    c.1920
 P  D  V  M  D  A  L  R   | S  S  W  A  G  K  E  S  T  L  K  R      p.640

           | 20        .         .         .         .         .    g.86050
 TCAGAGAAG | AGAGACAAAGAGTTCCTGAAGTCCACGTTTCTCCTGGTTTACCATGACTGC    c.1980
 S  E  K   | R  D  K  E  F  L  K  S  T  F  L  L  V  Y  H  D  C      p.660

          .         .         .         .         .         .       g.86110
 GTGCTCCCACTTCTCCACTCCACACGCCTACCCCCATTCAGGTGGGCAGAAGAAGAGACT       c.2040
 V  L  P  L  L  H  S  T  R  L  P  P  F  R  W  A  E  E  E  T         p.680

          .         .         .         .         .         .       g.86170
 GAGACTGCACGGTGGAAAGTTATCACTGACTTCCTTAAGCAAAACCAAGAAAACCAGGGC       c.2100
 E  T  A  R  W  K  V  I  T  D  F  L  K  Q  N  Q  E  N  Q  G         p.700

          .         .         .         .         .         .       g.86230
 GCCCTCCAAGCTCTGCTGTCACCAGACGGAGTTCATGAACCTTTTGACCTTTCAGAGCAG       c.2160
 A  L  Q  A  L  L  S  P  D  G  V  H  E  P  F  D  L  S  E  Q         p.720

          .         .         .         .                           g.86272
 ACCTATGACTTCTTGGGTGAAATGAGAAAGAATGCAGTGTGA                         c.2202
 T  Y  D  F  L  G  E  M  R  K  N  A  V  X                           p.733

          .         .         .         .         .         .       g.86332
 cagtggcagcctctagccctcagcttcccacggaatcagatggatcctccacgattacgt       c.*60

          .         .         .         .         .         .       g.86392
 gaataaaatgatggaaccaaaaatcactgtcactttacaacttaggttttactcttttct       c.*120

          .         .         .         .         .         .       g.86452
 ttctacagaccatatttttaaagaaatgtttatacaataatttaaatattttttaaaacc       c.*180

          .         .         .         .         .         .       g.86512
 ataaaataaatttttataaggaatactgttatatctaaatttaaacagtatttatttttt       c.*240

          .         .         .         .         .         .       g.86572
 caaaaacagctacttaagttaatggtatagatttctataaaagcaagattttgtcaaaaa       c.*300

          .         .         .         .         .         .       g.86632
 ctaaatttatgattattcaagaaagtgaaaaaaacaacctacagaatgggaaaacatatt       c.*360

          .         .         .         .         .         .       g.86692
 tgcaaatcatctaactgataaaggtctagtatccaaaatatttaaatttatgagtgttaa       c.*420

          .         .         .                                     g.86727
 taaaatttatcttgttcaatgaagaggaagttaaa                                c.*455

 (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 Nephronophthisis 1 (juvenile) 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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