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

(used for variant description)

(last modified January 23, 2022)


This file was created to facilitate the description of sequence variants on transcript NM_001128178.1 in the NPHP1 gene based on a coding DNA reference sequence following the HGVS recommendations.

The sequence was taken from NC_000002.11, covering NPHP1 transcript NM_001128178.1.


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         .         .         .         .  | 09      . g.46936
 AAGCAAAG | AACTGATCCCCACTGGAGTGCTGTTCAGAAAGCGATTTCAGAG | CAGATAAAC c.780
 K  Q  R  |  T  D  P  H  W  S  A  V  Q  K  A  I  S  E   | Q  I  N   p.260

          .         .         .         .         .         .       g.46996
 ACTGTTGATGTGTTAACTACGATGGGAGCTATTCCTGCAGGGTTCAGGCCTTCCACGCTC       c.840
 T  V  D  V  L  T  T  M  G  A  I  P  A  G  F  R  P  S  T  L         p.280

          .          | 10        .         .         .         .    g.48406
 TCACAGCTTCTGGAGGAAG | GGAATCAATTTCGAGCAAATTACTTCTTACAACCAGAGCTC    c.900
 S  Q  L  L  E  E  G |   N  Q  F  R  A  N  Y  F  L  Q  P  E  L      p.300

          .         .         .         .         .     | 11   .    g.49813
 ATGCCTTCACAACTGGCCTTCAGAGATCTGATGTGGGATGCTACAGAAGGCACT | ATTAGG    c.960
 M  P  S  Q  L  A  F  R  D  L  M  W  D  A  T  E  G  T   | I  R      p.320

          .         .         .         .         .         .       g.49873
 TCGAGACCAAGTCGTATTTCATTGATTCTGACATTATGGAGCTGTAAAATGATTCCTCTT       c.1020
 S  R  P  S  R  I  S  L  I  L  T  L  W  S  C  K  M  I  P  L         p.340

          .         .         .         .         .         .       g.49933
 CCAGGAATGAGCATACAGGTTCTCAGCAGACATGTACGCCTCTGTCTATTTGATGGTAAT       c.1080
 P  G  M  S  I  Q  V  L  S  R  H  V  R  L  C  L  F  D  G  N         p.360

     | 12    .         .         .         .         .         .    g.59863
 AAG | GTTCTGAGCAACATTCATACAGTCAGAGCCACATGGCAACCTAAAAAGCCCAAAACA    c.1140
 K   | V  L  S  N  I  H  T  V  R  A  T  W  Q  P  K  K  P  K  T      p.380

          .         | 13         .         .         .         .    g.62078
 TGGACCTTTTCTCCCCAG | GTTACTCGCATCTTACCATGTTTGCTTGATGGTGATTGCTTT    c.1200
 W  T  F  S  P  Q   | V  T  R  I  L  P  C  L  L  D  G  D  C  F      p.400

          .         .         .         .         .         .       g.62138
 ATCAGGTCTAATTCTGCATCTCCAGATCTTGGAATATTATTTGAACTTGGAATTTCTTAT       c.1260
 I  R  S  N  S  A  S  P  D  L  G  I  L  F  E  L  G  I  S  Y         p.420

           | 14        .         .         .         .         .    g.63278
 ATTCGCAAT | TCAACTGGTGAAAGAGGAGAGTTAAGCTGTGGCTGGGTGTTTCTTAAACTT    c.1320
 I  R  N   | S  T  G  E  R  G  E  L  S  C  G  W  V  F  L  K  L      p.440

          .         .         .   | 15     .         .         .    g.65521
 TTTGATGCCAGTGGAGTTCCTATTCCAGCAAA | AACTTATGAGCTTTTCTTGAATGGTGGT    c.1380
 F  D  A  S  G  V  P  I  P  A  K  |  T  Y  E  L  F  L  N  G  G      p.460

          .         .         .         .          | 16        .    g.66432
 ACTCCTTATGAAAAAGGTATTGAAGTGGACCCTTCAATATCCAGAAGAG | CACACGGCAGT    c.1440
 T  P  Y  E  K  G  I  E  V  D  P  S  I  S  R  R  A |   H  G  S      p.480

          .         .         .         .         .         .       g.66492
 GTTTTCTACCAGATTATGACAATGAGAAGGCAGCCTCAACTTCTAGTGAAACTGAGATCC       c.1500
 V  F  Y  Q  I  M  T  M  R  R  Q  P  Q  L  L  V  K  L  R  S         p.500

          .         .          | 17        .         .         .    g.78302
 TTGAACAGAAGATCAAGAAATGTACTAAG | TCTACTGCCAGAAACATTAATTGGAAATATG    c.1560
 L  N  R  R  S  R  N  V  L  S  |  L  L  P  E  T  L  I  G  N  M      p.520

          .         .         .         .         .         .       g.78362
 TGTTCTATTCACTTGTTGATATTTTATCGACAAATTCTTGGAGATGTGCTCCTGAAAGAC       c.1620
 C  S  I  H  L  L  I  F  Y  R  Q  I  L  G  D  V  L  L  K  D         p.540

          .         .   | 18     .         .         .         .    g.80841
 AGGATGAGCTTGCAAAGTACTG | ATTTAATTAGCCATCCCATGCTGGCCACCTTCCCCATG    c.1680
 R  M  S  L  Q  S  T  D |   L  I  S  H  P  M  L  A  T  F  P  M      p.560

          .         .         .       | 19 .         .         .    g.84405
 CTCTTGGAGCAGCCTGATGTGATGGATGCTCTCAGG | AGTTCGTGGGCTGGAAAAGAAAGC    c.1740
 L  L  E  Q  P  D  V  M  D  A  L  R   | S  S  W  A  G  K  E  S      p.580

          .         .  | 20      .         .         .         .    g.86038
 ACATTAAAAAGATCAGAGAAG | AGAGACAAAGAGTTCCTGAAGTCCACGTTTCTCCTGGTT    c.1800
 T  L  K  R  S  E  K   | R  D  K  E  F  L  K  S  T  F  L  L  V      p.600

          .         .         .         .         .         .       g.86098
 TACCATGACTGCGTGCTCCCACTTCTCCACTCCACACGCCTACCCCCATTCAGGTGGGCA       c.1860
 Y  H  D  C  V  L  P  L  L  H  S  T  R  L  P  P  F  R  W  A         p.620

          .         .         .         .         .         .       g.86158
 GAAGAAGAGACTGAGACTGCACGGTGGAAAGTTATCACTGACTTCCTTAAGCAAAACCAA       c.1920
 E  E  E  T  E  T  A  R  W  K  V  I  T  D  F  L  K  Q  N  Q         p.640

          .         .         .         .         .         .       g.86218
 GAAAACCAGGGCGCCCTCCAAGCTCTGCTGTCACCAGACGGAGTTCATGAACCTTTTGAC       c.1980
 E  N  Q  G  A  L  Q  A  L  L  S  P  D  G  V  H  E  P  F  D         p.660

          .         .         .         .         .                 g.86272
 CTTTCAGAGCAGACCTATGACTTCTTGGGTGAAATGAGAAAGAATGCAGTGTGA             c.2034
 L  S  E  Q  T  Y  D  F  L  G  E  M  R  K  N  A  V  X               p.677

          .         .         .         .         .         .       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.

Powered by LOVD v.3.0 Build 27
©2004-2022 Leiden University Medical Center