Meckel syndrome, type 1 (MKS1) - coding DNA reference sequence

(used for variant description)

(last modified November 29, 2023)


This file was created to facilitate the description of sequence variants on transcript NM_017777.3 in the MKS1 gene based on a coding DNA reference sequence following the HGVS recommendations.

The sequence was taken from NC_000017.10, covering MKS1 transcript NM_017777.3.


Please note that introns are available by clicking on the exon numbers above the sequence.
 (upstream sequence)
           .         .         .         .         .                g.5357
    ccgatccagcgtctccgttgccaggaaaacagagcggcgcgctttccggcgcagtcg       c.-2341

 .         .         .         .         .         .                g.5417
 cggcgcgtcgcagctgtcatggcggagaccgtctggagcactgacaccggggaggcagtg       c.-2281

 .         .         .         .        | 02.         .             g.5898
 tatcgctcccgggaccccgtgcgcaacttgcgcctccg | agtccacctgcaaagaatcaca    c.-2221

 .         .         .         .         .         .                g.5958
 tcaagcaactttcttcattatcagcctgctgccgagctcgggaaggacctcatagacttg       c.-2161

 .         .         .        | 03.         .         .             g.7901
 gccacttttaggcctcagccaactgcca | gtggacaccgcccagaggaagacgaagaggag    c.-2101

 .         .         .         .         | 04         .             g.8383
 gagattgtgattgggtggcaggagaagctctttagccag | tttgaagtagatctgtaccaa    c.-2041

 .         .         .         .         .         .                g.8443
 aatgaaacagcctgtcagagtcctttggattatcagtaccgtcaggagatcctgaagctg       c.-1981

 .         .         .         .         .         .                g.8503
 gagaattcgggtggcaagaaaaaccgacgaatctttacctacactgactctgatagatac       c.-1921

 .         .     | 05   .         .         .         .             g.9812
 accaatttggaggag | cactgtcagagaatgaccactgcagccagcgaggtgccttcattc    c.-1861

 .         .         .         .         .         .   | 06         g.10225
 ttggtcgagcgaatggcaaatgtcaggcgtcgccggcaggacaggcgagggat | ggagggc    c.-1801

 .         .         .         .         .         .                g.10285
 ggcatcctcaagtcacgcatcgtcacctgggagccctcagaagagtttgtcaggaacaac       c.-1741

 .         .         .         .         .         .                g.10345
 cacgtcattaacacccctcttcagacaatgcacatcatggcagacctggggccctataaa       c.-1681

 .  | 07      .         .         .         .         .             g.10794
 aa | gcttggctataagaagtatgaacatgtcctgtgtactctgaaggtggatagcaatggt    c.-1621

 .         .         .         .         .       | 08 .             g.11528
 gtgatcacagtaaagcctgacttcacgggcctcaaaggaccctacag | gattgagacggag    c.-1561

 .         .         .         .         .         .                g.11588
 ggggagaagcaggagctgtggaaatatacgatcgacaatgtttccccccacgcacagccg       c.-1501

 .         .         .         .      | 09  .         .             g.12195
 gaggaggaggagcgggaacggcgagtgttcaaggat | ctttatggccggcacaaggagtat    c.-1441

 .         .         .         .   | 10     .         .             g.13610
 ctcagcagcctcgtaggcaccgactttgagatg | actgtcccaggtgccctccggctcttt    c.-1381

 .         .      | 11  .         .         .         .             g.13925
 gtaaatggagaggtcg | tttcagcccaaggctatgagtatgacaatctctacgtccacttc    c.-1321

 .         .         .  | 12      .         .         .             g.16060
 tttgtagaattgccaactgctc | actggtcaagcccagcattccagcagctctcaggagta    c.-1261

 .         .         .         .   | 13     .         .             g.16458
 acacagacctgcaccaccaagtccctggcaatg | gacaaggtggctcacttctcctaccca    c.-1201

 .         .         .         .         .   | 14     .             g.16621
 ttcacgtttgaagccttcttcctccatgaggatgaatcttctg | atgcactcccggagtgg    c.-1141

 .         .         .         .         .         .                g.16681
 cctgtgctctactgtgaggtcctctcgctggacttctggcagaggtaccgtgtggaaggc       c.-1081

 .         .         .         . | 15       .         .             g.17416
 tatggggctgtggtgctgcctgccactccag | gctcacacaccctgacagtctccacgtgg    c.-1021

 .         .         .         .         .         .                g.17476
 agacctgtggagcttggcacggtggctgagctgaggaggtttttcattggcggttctctg       c.-961

 .         .         .         .         .     | 16   .             g.18073
 gaactggaggacctctcctatgtacggataccaggatccttcaag | ggggaacgcctgagc    c.-901

 .         .         .         .         .         .                g.18133
 cgctttggactccgcacagagaccacaggcactgtcaccttccgcttgcactgtctgcag       c.-841

 .        | 17.         .         .         .         .             g.18277
 cagtccag | ggccttcatggaatcgagctcccttcagaaaaggatgcggagtgtgttggac    c.-781

 .         .         .         .         .      | 18  .             g.18449
 cgtctggaagggttcagccagcagagttccattcacaatgtgctag | aggccttccgtcga    c.-721

 .         .         .         .         .         .                g.18509
 gcccggcgccgcatgcaggaggcccgggaaagcctcccgcaggacctagtgagcccctct       c.-661

 .         .         .         .         .         .                g.18569
 ggaaccctggtctcctagctcacagcagccctggcccacagtgcaagaggacaagatggg       c.-601

 .         .         .         .         .         .                g.18629
 ggatatctgaggccagtgctctcctgcctcttcgtctttctgattagagaccacgttggt       c.-541

 .         .         .         .         .         .                g.18689
 ctgctgagaaccagaaaagctgggaaattcccggctggcccctctgcctagccttctgca       c.-481

 .         .         .         .         .         .                g.18749
 gggtcttctccctaccctgtggaaataaagccagagaccctgtggccccccctttatatt       c.-421

 .         .         .         .         .         .                g.18809
 tgcagccactgtcaatgggcatcacagcaaggcaggctttttgatctgaagctgtttggg       c.-361

 .         .         .         .         .         .                g.18869
 gtgaccccatagtgagtctggccttgactcacttttctggcttgtctcacagtagcaggg       c.-301

 .         .         .         .         .         .                g.18929
 gatctgctgttctccaccctttaaaaatggagcctttcatcttccaagaccaaagaagga       c.-241

 .         .         .         .         .         .                g.18989
 aggaaggtggacggtgagagggtcttaaatccccagcctgtgagtctcagcctctttcca       c.-181

 .         .         .         .         .         .                g.19049
 gggaagcctcattctactgcttggggcttgctgaggttgagatatgagttatttctgagt       c.-121

 .         .         .         .         .         .                g.19109
 aagtgaaggctgaaggcagtgctgagtcctttctgctagctgggtggtatcttttactgt       c.-61

 .         .         .         .         .         .                g.19169
 gtttttttgtttgtttgtttttttgttttttgttacataaaccccttttattttgactga       c.-1

          .         .         .         .         .         .       g.19229
 CCGATCCAGCGTCTCCGTTGCCAGGAAAACAGAGCGGCGCGCTTTCCGGCGCAGTCGCGG       c.60
 P  I  Q  R  L  R  C  Q  E  N  R  A  A  R  F  P  A  Q  S  R         p.20

          .         .         .         .         .         .       g.19289
 CGCGTCGCAGCTGTCATGGCGGAGACCGTCTGGAGCACTGACACCGGGGAGGCAGTGTAT       c.120
 R  V  A  A  V  M  A  E  T  V  W  S  T  D  T  G  E  A  V  Y         p.40

          .         .         .      | 19  .         .         .    g.19349
 CGCTCCCGGGACCCCGTGCGCAACTTGCGCCTCCG | AGTCCACCTGCAAAGAATCACATCA    c.180
 R  S  R  D  P  V  R  N  L  R  L  R  |  V  H  L  Q  R  I  T  S      p.60

          .         .         .         .         .         .       g.19409
 AGCAACTTTCTTCATTATCAGCCTGCTGCCGAGCTCGGGAAGGACCTCATAGACTTGGCC       c.240
 S  N  F  L  H  Y  Q  P  A  A  E  L  G  K  D  L  I  D  L  A         p.80

          .         .      | 20  .         .         .         .    g.19469
 ACTTTTAGGCCTCAGCCAACTGCCA | GTGGACACCGCCCAGAGGAAGACGAAGAGGAGGAG    c.300
 T  F  R  P  Q  P  T  A  S |   G  H  R  P  E  E  D  E  E  E  E      p.100

          .         .         .       | 21 .         .         .    g.19529
 ATTGTGATTGGGTGGCAGGAGAAGCTCTTTAGCCAG | TTTGAAGTAGATCTGTACCAAAAT    c.360
 I  V  I  G  W  Q  E  K  L  F  S  Q   | F  E  V  D  L  Y  Q  N      p.120

          .         .         .         .         .         .       g.19589
 GAAACAGCCTGTCAGAGTCCTTTGGATTATCAGTACCGTCAGGAGATCCTGAAGCTGGAG       c.420
 E  T  A  C  Q  S  P  L  D  Y  Q  Y  R  Q  E  I  L  K  L  E         p.140

          .         .         .         .         .         .       g.19649
 AATTCGGGTGGCAAGAAAAACCGACGAATCTTTACCTACACTGACTCTGATAGATACACC       c.480
 N  S  G  G  K  K  N  R  R  I  F  T  Y  T  D  S  D  R  Y  T         p.160

          .   | 22     .         .         .         .         .    g.19709
 AATTTGGAGGAG | CACTGTCAGAGAATGACCACTGCAGCCAGCGAGGTGCCTTCATTCTTG    c.540
 N  L  E  E   | H  C  Q  R  M  T  T  A  A  S  E  V  P  S  F  L      p.180

          .         .         .         .         . | 23       .    g.19769
 GTCGAGCGAATGGCAAATGTCAGGCGTCGCCGGCAGGACAGGCGAGGGAT | GGAGGGCGGC    c.600
 V  E  R  M  A  N  V  R  R  R  R  Q  D  R  R  G  M  |  E  G  G      p.200

          .         .         .         .         .         .       g.19829
 ATCCTCAAGTCACGCATCGTCACCTGGGAGCCCTCAGAAGAGTTTGTCAGGAACAACCAC       c.660
 I  L  K  S  R  I  V  T  W  E  P  S  E  E  F  V  R  N  N  H         p.220

          .         .         .         .         .          | 24    g.19889
 GTCATTAACACCCCTCTTCAGACAATGCACATCATGGCAGACCTGGGGCCCTATAAAAA | G    c.720
 V  I  N  T  P  L  Q  T  M  H  I  M  A  D  L  G  P  Y  K  K  |      p.240

          .         .         .         .         .         .       g.19949
 CTTGGCTATAAGAAGTATGAACATGTCCTGTGTACTCTGAAGGTGGATAGCAATGGTGTG       c.780
 L  G  Y  K  K  Y  E  H  V  L  C  T  L  K  V  D  S  N  G  V         p.260

          .         .         .         .     | 25   .         .    g.20009
 ATCACAGTAAAGCCTGACTTCACGGGCCTCAAAGGACCCTACAG | GATTGAGACGGAGGGG    c.840
 I  T  V  K  P  D  F  T  G  L  K  G  P  Y  R  |  I  E  T  E  G      p.280

          .         .         .         .         .         .       g.20069
 GAGAAGCAGGAGCTGTGGAAATATACGATCGACAATGTTTCCCCCCACGCACAGCCGGAG       c.900
 E  K  Q  E  L  W  K  Y  T  I  D  N  V  S  P  H  A  Q  P  E         p.300

          .         .         .    | 26    .         .         .    g.20129
 GAGGAGGAGCGGGAACGGCGAGTGTTCAAGGAT | CTTTATGGCCGGCACAAGGAGTATCTC    c.960
 E  E  E  R  E  R  R  V  F  K  D   | L  Y  G  R  H  K  E  Y  L      p.320

          .         .         . | 27       .         .         .    g.20189
 AGCAGCCTCGTAGGCACCGACTTTGAGATG | ACTGTCCCAGGTGCCCTCCGGCTCTTTGTA    c.1020
 S  S  L  V  G  T  D  F  E  M   | T  V  P  G  A  L  R  L  F  V      p.340

          .    | 28    .         .         .         .         .    g.20249
 AATGGAGAGGTCG | TTTCAGCCCAAGGCTATGAGTATGACAATCTCTACGTCCACTTCTTT    c.1080
 N  G  E  V  V |   S  A  Q  G  Y  E  Y  D  N  L  Y  V  H  F  F      p.360

          .          | 29        .         .         .         .    g.20309
 GTAGAATTGCCAACTGCTC | ACTGGTCAAGCCCAGCATTCCAGCAGCTCTCAGGAGTAACA    c.1140
 V  E  L  P  T  A  H |   W  S  S  P  A  F  Q  Q  L  S  G  V  T      p.380

          .         .         . | 30       .         .         .    g.20369
 CAGACCTGCACCACCAAGTCCCTGGCAATG | GACAAGGTGGCTCACTTCTCCTACCCATTC    c.1200
 Q  T  C  T  T  K  S  L  A  M   | D  K  V  A  H  F  S  Y  P  F      p.400

          .         .         .         . | 31       .         .    g.20429
 ACGTTTGAAGCCTTCTTCCTCCATGAGGATGAATCTTCTG | ATGCACTCCCGGAGTGGCCT    c.1260
 T  F  E  A  F  F  L  H  E  D  E  S  S  D |   A  L  P  E  W  P      p.420

          .         .         .         .         .         .       g.20489
 GTGCTCTACTGTGAGGTCCTCTCGCTGGACTTCTGGCAGAGGTACCGTGTGGAAGGCTAT       c.1320
 V  L  Y  C  E  V  L  S  L  D  F  W  Q  R  Y  R  V  E  G  Y         p.440

          .         .         | 32         .         .         .    g.20549
 GGGGCTGTGGTGCTGCCTGCCACTCCAG | GCTCACACACCCTGACAGTCTCCACGTGGAGA    c.1380
 G  A  V  V  L  P  A  T  P  G |   S  H  T  L  T  V  S  T  W  R      p.460

          .         .         .         .         .         .       g.20609
 CCTGTGGAGCTTGGCACGGTGGCTGAGCTGAGGAGGTTTTTCATTGGCGGTTCTCTGGAA       c.1440
 P  V  E  L  G  T  V  A  E  L  R  R  F  F  I  G  G  S  L  E         p.480

          .         .         .         .   | 33     .         .    g.20669
 CTGGAGGACCTCTCCTATGTACGGATACCAGGATCCTTCAAG | GGGGAACGCCTGAGCCGC    c.1500
 L  E  D  L  S  Y  V  R  I  P  G  S  F  K   | G  E  R  L  S  R      p.500

          .         .         .         .         .         .       g.20729
 TTTGGACTCCGCACAGAGACCACAGGCACTGTCACCTTCCGCTTGCACTGTCTGCAGCAG       c.1560
 F  G  L  R  T  E  T  T  G  T  V  T  F  R  L  H  C  L  Q  Q         p.520

       | 34  .         .         .         .         .         .    g.20789
 TCCAG | GGCCTTCATGGAATCGAGCTCCCTTCAGAAAAGGATGCGGAGTGTGTTGGACCGT    c.1620
 S  R  |  A  F  M  E  S  S  S  L  Q  K  R  M  R  S  V  L  D  R      p.540

          .         .         .         .    | 35    .         .    g.20849
 CTGGAAGGGTTCAGCCAGCAGAGTTCCATTCACAATGTGCTAG | AGGCCTTCCGTCGAGCC    c.1680
 L  E  G  F  S  Q  Q  S  S  I  H  N  V  L  E |   A  F  R  R  A      p.560

          .         .         .         .         .         .       g.20909
 CGGCGCCGCATGCAGGAGGCCCGGGAAAGCCTCCCGCAGGACCTAGTGAGCCCCTCTGGA       c.1740
 R  R  R  M  Q  E  A  R  E  S  L  P  Q  D  L  V  S  P  S  G         p.580

          .                                                         g.20924
 ACCCTGGTCTCCTAG                                                    c.1755
 T  L  V  S  X                                                      p.584

          .         .         .         .         .         .       g.20984
 ctcacagcagccctggcccacagtgcaagaggacaagatgggggatatctgaggccagtg       c.*60

          .         .         .         .         .         .       g.21044
 ctctcctgcctcttcgtctttctgattagagaccacgttggtctgctgagaaccagaaaa       c.*120

          .         .         .         .         .         .       g.21104
 gctgggaaattcccggctggcccctctgcctagccttctgcagggtcttctccctaccct       c.*180

          .         .         .         .         .         .       g.21164
 gtggaaataaagccagagaccctgtggccccccctttatatttgcagccactgtcaatgg       c.*240

          .         .         .         .         .         .       g.21224
 gcatcacagcaaggcaggctttttgatctgaagctgtttggggtgaccccatagtgagtc       c.*300

          .         .         .         .         .         .       g.21284
 tggccttgactcacttttctggcttgtctcacagtagcaggggatctgctgttctccacc       c.*360

          .         .         .         .         .         .       g.21344
 ctttaaaaatggagcctttcatcttccaagaccaaagaaggaaggaaggtggacggtgag       c.*420

          .         .         .         .         .         .       g.21404
 agggtcttaaatccccagcctgtgagtctcagcctctttccagggaagcctcattctact       c.*480

          .         .         .         .         .         .       g.21464
 gcttggggcttgctgaggttgagatatgagttatttctgagtaagtgaaggctgaaggca       c.*540

          .         .         .         .         .         .       g.21524
 gtgctgagtcctttctgctagctgggtggtatcttttactgtgtttttttgtttgtttgt       c.*600

          .         .         .         .                           g.21567
 ttttttgttttttgttacataaaccccttttattttgactgat                        c.*643

 (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 Meckel syndrome, type 1 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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