Ellis van Creveld syndrome 2 (EVC2) - coding DNA reference sequence

(used for variant description)

(last modified January 3, 2021)


This file was created to facilitate the description of sequence variants on transcript NM_147127.4 in the EVC2 gene based on a coding DNA reference sequence following the HGVS recommendations.

The sequence was taken from NG_015821.1, covering EVC2 transcript NM_147127.4.


Please note that introns are available by clicking on the exon numbers above the sequence.
 (upstream sequence)
           .         .         .         .         .                g.6035
       gctccagcgactcggcggcacccgccgctttgaggtagcgggtcccgacaggcg       c.-1

          .         .         .         .         .         .       g.6095
 ATGGACCCCTCGGGCTCCCGGGGGCGCCCCACGTGGGTGCTGGCCGGGGGTCTCCTGGCA       c.60
 M  D  P  S  G  S  R  G  R  P  T  W  V  L  A  G  G  L  L  A         p.20

          .         .         .         .         .         .       g.6155
 GTGGCCCTGGCGCTGGGGGGCCGAGGCTGTCTCGGCGCCAGCTCACGTCCCCGCTGGCGC       c.120
 V  A  L  A  L  G  G  R  G  C  L  G  A  S  S  R  P  R  W  R         p.40

          .         .         .         .         .         .       g.6215
 CCCCTCGGCGCGCAGCCACCCCGGGATCCCCAGGTGGCTCCTAGGTCTGGGCCCGGCCTG       c.180
 P  L  G  A  Q  P  P  R  D  P  Q  V  A  P  R  S  G  P  G  L         p.60

          .         .         .         .         | 02         .    g.16913
 AGGATCCCTCCGGGGCGGAGCGGGGCGGGGCCCGAGAGCAGCACGCAG | GACTTGCCCTGT    c.240
 R  I  P  P  G  R  S  G  A  G  P  E  S  S  T  Q   | D  L  P  C      p.80

          .         .         .         .    | 03    .         .    g.20064
 ATGATTTGGCCCAAAGTGGAATGCTGTCACTTTAAGACTGCAG | TGGAAGCACCACTTGGA    c.300
 M  I  W  P  K  V  E  C  C  H  F  K  T  A  V |   E  A  P  L  G      p.100

          .         .         .         .         .         .       g.20124
 ATGAAATTGGACAAGAAAATGGAAGTCTTCATCCCACTCTCAACTTCTGCAGCCTCTAGT       c.360
 M  K  L  D  K  K  M  E  V  F  I  P  L  S  T  S  A  A  S  S         p.120

          .         .         .         .         .         .       g.20184
 GGGCCATGGGCTCATTCCTTATTTGCTTTTATACCCTCCTGGCCTAAGAAGAACTTATTT       c.420
 G  P  W  A  H  S  L  F  A  F  I  P  S  W  P  K  K  N  L  F         p.140

          .         .         . | 04       .         .         .    g.23245
 AAAAGAGAGTCTCCTATAACACACCGCCTG | TATGGGGACATTTCAAGAGAAGTTCAAGGG    c.480
 K  R  E  S  P  I  T  H  R  L   | Y  G  D  I  S  R  E  V  Q  G      p.160

          .         .         .          | 05        .         .    g.25226
 ACTTCTGAAAATGGAGTAATTTTTCAGAAATGTGCACTG | GTGTCTGGGTCGAGTGAAGCA    c.540
 T  S  E  N  G  V  I  F  Q  K  C  A  L   | V  S  G  S  S  E  A      p.180

          .         .         .         .         .         .       g.25286
 CAGACAGCCCGCATATGGCTGCTTGTTAACAACACCAAGACAACCTCGTCAGCCAACCTC       c.600
 Q  T  A  R  I  W  L  L  V  N  N  T  K  T  T  S  S  A  N  L         p.200

          .         .         .         .         .         .       g.25346
 TCGGAGCTGCTCTTGCTGGACAGCATTGCTGGTCTCACCATTTGGGACTCTGTGGGAAAC       c.660
 S  E  L  L  L  L  D  S  I  A  G  L  T  I  W  D  S  V  G  N         p.220

          .         .         .         .       | 06 .         .    g.29083
 AGGACCTCGGAAGGATTCCAGGCTTTTAGCAAGAAGTTTCTGCAAG | TGGGAGATGCCTTT    c.720
 R  T  S  E  G  F  Q  A  F  S  K  K  F  L  Q  V |   G  D  A  F      p.240

          .         .         .         .         .         .       g.29143
 GCTGTCAGCTACGCAGCCACGCTCCAGGCTGGAGACCTCGGGAACGGGGAGAGCCTCAAG       c.780
 A  V  S  Y  A  A  T  L  Q  A  G  D  L  G  N  G  E  S  L  K         p.260

          .         .         .       | 07 .         .         .    g.33259
 CTTCCTGCCCAACTCACCTTTCAGAGCTCGTCACGG | AACAGAACACAGCTGAAAGTGCTT    c.840
 L  P  A  Q  L  T  F  Q  S  S  S  R   | N  R  T  Q  L  K  V  L      p.280

          .         .         . | 08       .         .         .    g.48929
 TTTTCCATAACAGCAGAAGAAAACGTAACG | GTTCTGCCGCACCACGGCCTCCACGCAGCA    c.900
 F  S  I  T  A  E  E  N  V  T   | V  L  P  H  H  G  L  H  A  A      p.300

          .         .         .         .         .         .       g.48989
 GGGTTCTTCATTGCCTTCCTCCTCTCCCTTGTGCTGACCTGGGCTGCCCTCTTCCTCATG       c.960
 G  F  F  I  A  F  L  L  S  L  V  L  T  W  A  A  L  F  L  M         p.320

          .         .         .         .      | 09  .         .    g.51317
 GTTCGCTATCAGTGTCTGAAGGGAAACATGCTCACCAGACATCGG | GTTTGGCAGTATGAG    c.1020
 V  R  Y  Q  C  L  K  G  N  M  L  T  R  H  R   | V  W  Q  Y  E      p.340

          .         .         .         .         .         .       g.51377
 AGCAAGCTGGAACCCTTGCCGTTCACCTCAGCTGATGGCGTGAATGAGGACCTTTCCCTT       c.1080
 S  K  L  E  P  L  P  F  T  S  A  D  G  V  N  E  D  L  S  L         p.360

          .         .         .         .         .         .       g.51437
 AACGACCAAATGATAGACATTCTGTCTTCCGAGGACCCTGGGAGCATGCTTCAAGCCTTA       c.1140
 N  D  Q  M  I  D  I  L  S  S  E  D  P  G  S  M  L  Q  A  L         p.380

       | 10  .         .         .         .         .         .    g.73765
 GAAGA | GTTGGAGATTGCAACCCTGAATCGGGCAGATGCAGATCTGGAGGCTTGTCGAACA    c.1200
 E  E  |  L  E  I  A  T  L  N  R  A  D  A  D  L  E  A  C  R  T      p.400

          .         .         .         .         .         .       g.73825
 CAAATCAGCAAGGATATCATTGCCCTTCTGCTGAAAAATCTCACCAGCAGTGGCCACCTC       c.1260
 Q  I  S  K  D  I  I  A  L  L  L  K  N  L  T  S  S  G  H  L         p.420

          .         .         .         .         .         .       g.73885
 TCACCCCAAGTAGAGAGAAAAATGAGTGCTGTTTTCAAAAAGCAGTTTCTATTGCTGGAA       c.1320
 S  P  Q  V  E  R  K  M  S  A  V  F  K  K  Q  F  L  L  L  E         p.440

          .         .         .         .         .         .       g.73945
 AATGAAATACAAGAGGAGTACGATCGGAAGATGGTGGCATTGACAGCTGAATGTGACCTG       c.1380
 N  E  I  Q  E  E  Y  D  R  K  M  V  A  L  T  A  E  C  D  L         p.460

          .         .         .         .         .         .       g.74005
 GAAACAAGAAAGAAGATGGAAAACCAGTACCAGAGAGAGATGATGGCAATGGAGGAAGCA       c.1440
 E  T  R  K  K  M  E  N  Q  Y  Q  R  E  M  M  A  M  E  E  A         p.480

          .         .         . | 11       .         .         .    g.82546
 GAAGAGTTGCTGAAACGTGCTGGTGAGAGG | TCTGCTGTAGAGTGCAGCAACCTTCTGCGG    c.1500
 E  E  L  L  K  R  A  G  E  R   | S  A  V  E  C  S  N  L  L  R      p.500

          .         .         .         .         .         .       g.82606
 ACCCTCCATGGCCTGGAACAGGAGCACTTGAGGAAGTCTCTCGCTTTGCAACAAGAAGAA       c.1560
 T  L  H  G  L  E  Q  E  H  L  R  K  S  L  A  L  Q  Q  E  E         p.520

          .         .         .         .         .         .       g.82666
 GACTTTGCCAAAGCTCACAGACAGCTGGCTGTTTTCCAGAGAAATGAACTGCACAGTATC       c.1620
 D  F  A  K  A  H  R  Q  L  A  V  F  Q  R  N  E  L  H  S  I         p.540

          .         .         .         .         .         .       g.82726
 TTTTTTACCCAGATAAAAAGTGCTATTTTCAAAGGGGAATTGAAACCAGAGGCAGCTAAA       c.1680
 F  F  T  Q  I  K  S  A  I  F  K  G  E  L  K  P  E  A  A  K         p.560

          .         .         . | 12       .         .         .    g.85844
 ATGCTGCTGCAAAATTATTCTAAAATACAG | GAGAATGTAGAAGAGTTAATGGACTTTTTC    c.1740
 M  L  L  Q  N  Y  S  K  I  Q   | E  N  V  E  E  L  M  D  F  F      p.580

          .         .         .         .         .         .       g.85904
 CAGGCTAGTAAGAGGTATCATCTAAGTAAAAGATTTGGCCACAGGGAATATCTGGTCCAG       c.1800
 Q  A  S  K  R  Y  H  L  S  K  R  F  G  H  R  E  Y  L  V  Q         p.600

          .         .         .         .         .         .       g.85964
 AACCTCCAGTCATCAGAGACCCGTGTGCAGGGCCTTCTGAGCACCGCTGCAGCCCAGCTG       c.1860
 N  L  Q  S  S  E  T  R  V  Q  G  L  L  S  T  A  A  A  Q  L         p.620

          .         .       | 13 .         .         .         .    g.88674
 ACTCACCTCATTCAGAAGCACGAGAG | AGCAGGGTACCTGGATGAAGACCAAATGGAAATG    c.1920
 T  H  L  I  Q  K  H  E  R  |  A  G  Y  L  D  E  D  Q  M  E  M      p.640

          .         .         .         .         .         .       g.88734
 CTATTGGAGCGGGCTCAGACAGAAGTCTTTTCAATCAAGCAGAAGTTGGACAATGACTTA       c.1980
 L  L  E  R  A  Q  T  E  V  F  S  I  K  Q  K  L  D  N  D  L         p.660

          .         .         .         .         .         .       g.88794
 AAGCAGGAAAAGAAAAAGCTCCACCAAAAATTAATAACTAAGAGAAGACGAGAGTTGCTA       c.2040
 K  Q  E  K  K  K  L  H  Q  K  L  I  T  K  R  R  R  E  L  L         p.680

        | 14 .         .         .         .         .         .    g.91611
 CAAAAG | CACAGGGAGCAGCGTAGGGAGCAGGCGTCCGTCGGCGAGGCCTTCCGAACGGTT    c.2100
 Q  K   | H  R  E  Q  R  R  E  Q  A  S  V  G  E  A  F  R  T  V      p.700

          .         .         .         .         .         .       g.91671
 GAGGATGCCGGCCAGTACCTGCACCAGAAGAGGAGCCTGATGGAGGAGCACGGTGCCACC       c.2160
 E  D  A  G  Q  Y  L  H  Q  K  R  S  L  M  E  E  H  G  A  T         p.720

          .         .         .         .         .         .       g.91731
 CTGGAGGAGCTGCAGGAGCGTCTGGACCAGGCCGCCCTGGACGATCTCAGGACCCTGACC       c.2220
 L  E  E  L  Q  E  R  L  D  Q  A  A  L  D  D  L  R  T  L  T         p.740

          .         .         .         .         .         .       g.91791
 CTTTCGCTGTTTGAAAAGGCCACCGACGAGCTGCGGCGCCTGCAGAACTCAGCCATGACC       c.2280
 L  S  L  F  E  K  A  T  D  E  L  R  R  L  Q  N  S  A  M  T         p.760

          .         .         .         .         .         .       g.91851
 CAGGAGCTGCTCAAGCGTGGGGTGCCCTGGCTCTTCCTGCAGCAGATCCTGGAGGAGCAC       c.2340
 Q  E  L  L  K  R  G  V  P  W  L  F  L  Q  Q  I  L  E  E  H         p.780

          .         .         .         .         .         .       g.91911
 GGCAAGGAGATGGCTGCACGGGCCGAGCAGCTGGAGGGGGAGGAGAGGGACAGGGACCAG       c.2400
 G  K  E  M  A  A  R  A  E  Q  L  E  G  E  E  R  D  R  D  Q         p.800

          .         .         .         .         .         .       g.91971
 GAGGGTGTCCAGAGCGTGAGGCAGAGACTGAAGGATGACGCTCCTGAGGCCGTGACAGAG       c.2460
 E  G  V  Q  S  V  R  Q  R  L  K  D  D  A  P  E  A  V  T  E         p.820

          .         .         .         .  | 15      .         .    g.95885
 GAGCAGGCAGAGCTGCGACGCTGGGAGCACCTGATCTTCAT | GAAGCTCTGCTCCTCAGTC    c.2520
 E  Q  A  E  L  R  R  W  E  H  L  I  F  M  |  K  L  C  S  S  V      p.840

          .         .         .         .         .         .       g.95945
 TTCTCCCTGTCTGAAGAGGAGCTGCTCAGGATGAGGCAGGAGGTCCATGGCTGCTTTGCT       c.2580
 F  S  L  S  E  E  E  L  L  R  M  R  Q  E  V  H  G  C  F  A         p.860

          .         .         .         .         .         .       g.96005
 CAGATGGACAGGAGCTTGGCCCTCCCCAAGATCCGGGCCCGAGTTCTGCTGCAGCAATTT       c.2640
 Q  M  D  R  S  L  A  L  P  K  I  R  A  R  V  L  L  Q  Q  F         p.880

          .         .         .         .         .         .       g.96065
 CAGACTGCGTGGCGAGAAGCAGAGTTCGTGAAGCTGGACCAGGCCGTGGCTGCCCCTGAG       c.2700
 Q  T  A  W  R  E  A  E  F  V  K  L  D  Q  A  V  A  A  P  E         p.900

        | 16 .         .         .         .         .         .    g.99058
 CTGCAG | CAACAGTCCAAGGTGAGAAAGTCACGGTCCAAGAGTAAAAGCAAGGGAGAGCTT    c.2760
 L  Q   | Q  Q  S  K  V  R  K  S  R  S  K  S  K  S  K  G  E  L      p.920

          .         .         .         .         .         .       g.99118
 CTGAAGAAGTGCATCGAAGACAAAATTCACCTCTGTGAGGAACAGGCCTCTGAAGACCTG       c.2820
 L  K  K  C  I  E  D  K  I  H  L  C  E  E  Q  A  S  E  D  L         p.940

           | 17        .         .         .         .         .    g.129749
 GTGGAAAAG | GTTCGAGGTGAATTGCTGCGGGAGAGAGTGCAGCGGATGGAGGCACAGGAG    c.2880
 V  E  K   | V  R  G  E  L  L  R  E  R  V  Q  R  M  E  A  Q  E      p.960

          .         .         .         .         .         .       g.129809
 GGAGGCTTTGCACAGTCGCTTGTTGCTCTGCAGTTCCAGAAGGCGTCCCGGGTGACCGAG       c.2940
 G  G  F  A  Q  S  L  V  A  L  Q  F  Q  K  A  S  R  V  T  E         p.980

          .         .         .         .         .         .       g.129869
 ACTCTGTCGGCCTACACCGCCCTCCTCAGCATCCAGGACTTGCTCCTGGAAGAGCTGAGT       c.3000
 T  L  S  A  Y  T  A  L  L  S  I  Q  D  L  L  L  E  E  L  S         p.1000

          .         .         .         .         .        | 18.    g.138097
 GCATCTGAGATGCTGACCAAGTCGGCCTGCACACAGATCCTGGAGTCGCACAGCCGG | GAG    c.3060
 A  S  E  M  L  T  K  S  A  C  T  Q  I  L  E  S  H  S  R   | E      p.1020

          .         .         .         .         .         .       g.138157
 CTCCAGGAGTTGGAGAGGAAGCTGGAGGACCAGCTGGTGCAGCAGGAGGCAGCCCAGCAG       c.3120
 L  Q  E  L  E  R  K  L  E  D  Q  L  V  Q  Q  E  A  A  Q  Q         p.1040

          .         .         .         .         .         .       g.138217
 CAGCAGGCCCTGGCGAGCTGGCAGCAGTGGGTGGCCGATGGGCCCGGGATTCTGAACGAA       c.3180
 Q  Q  A  L  A  S  W  Q  Q  W  V  A  D  G  P  G  I  L  N  E         p.1060

          .         .         .         .         .         .       g.138277
 CCTGGGGAGGTGGATTCTGAAAGGCAGGTCTCTACTGTCCTGCACCAAGCCCTGAGCAAG       c.3240
 P  G  E  V  D  S  E  R  Q  V  S  T  V  L  H  Q  A  L  S  K         p.1080

          .         .         .   | 19     .         .         .    g.139804
 AGCCAGACATTACTGGAGCAACATCAGCAGTG | TTTGAGAGAGGAACAACAGAACAGTGTC    c.3300
 S  Q  T  L  L  E  Q  H  Q  Q  C  |  L  R  E  E  Q  Q  N  S  V      p.1100

          .         .         .         .         .         .       g.139864
 GTGCTAGAAGACTTGTTGGAAAACATGGAGGCAGACACCTTTGCAACCCTGTGCAGCCAG       c.3360
 V  L  E  D  L  L  E  N  M  E  A  D  T  F  A  T  L  C  S  Q         p.1120

  | 20       .         .         .         .         .         .    g.145968
  | GAGCTGAGACTGGCATCGTACCTGGCGAGGATGGCCATGGTGCCCGGGGCCACGCTTCGC    c.3420
  | E  L  R  L  A  S  Y  L  A  R  M  A  M  V  P  G  A  T  L  R      p.1140

          .         .         .         .         .         .       g.146028
 CGGCTCCTGAGTGTGGTACTGCCCACAGCCTCACAGCCTCAGCTGCTGGCCCTGCTGGAT       c.3480
 R  L  L  S  V  V  L  P  T  A  S  Q  P  Q  L  L  A  L  L  D         p.1160

          .         .         .         .         .         .       g.146088
 TCGGCCACCGAGAGACATGTGGACCACGCAGCTGAGAGCGATGGCGGAGCGGAGCAGGCC       c.3540
 S  A  T  E  R  H  V  D  H  A  A  E  S  D  G  G  A  E  Q  A         p.1180

          .        | 21.         .         .         .         .    g.149232
 GACGTGGGCAGGCGGAG | GAAACACCAGAGCTGGTGGCAAGCCTTAGATGGCAAACTGCGA    c.3600
 D  V  G  R  R  R  |  K  H  Q  S  W  W  Q  A  L  D  G  K  L  R      p.1200

          .         .         .         .         .          | 22    g.151434
 GGAGATCTGATAAGCAGAGGATTAGAAAAGATGCTGTGGGCCCGCAAGAGAAAGCAGAG | C    c.3660
 G  D  L  I  S  R  G  L  E  K  M  L  W  A  R  K  R  K  Q  S  |      p.1220

          .         .         .         .         .         .       g.151494
 ATATTAAAGAAGACATGTCTCCCTCTCAGAGAGAGGATGATATTCTCTGGAAAAGGAAGT       c.3720
 I  L  K  K  T  C  L  P  L  R  E  R  M  I  F  S  G  K  G  S         p.1240

          .         .         .         .         .         .       g.151554
 TGGCCACACCTGTCACTGGAGCCCATTGGCGAACTGGCCCCTGTACCCATTGTAGGGGCA       c.3780
 W  P  H  L  S  L  E  P  I  G  E  L  A  P  V  P  I  V  G  A         p.1260

          .         .         .         .         .         .       g.151614
 GAAACCATTGATCTATTAAACACAGGAGAGAAGCTCTTTATATTCAGAAATCCAAAGGAG       c.3840
 E  T  I  D  L  L  N  T  G  E  K  L  F  I  F  R  N  P  K  E         p.1280

          .         .         .         .         .         .       g.151674
 CCAGAGATCTCACTGCACGTTCCTCCCAGGAAAAAGAAGAACTTTTTGAATGCCAAAAAG       c.3900
 P  E  I  S  L  H  V  P  P  R  K  K  K  N  F  L  N  A  K  K         p.1300

          .         .                                               g.151701
 GCCATGAGGGCCTTGGGCATGGACTAG                                        c.3927
 A  M  R  A  L  G  M  D  X                                          p.1308

          .         .         .         .         .         .       g.151761
 cccaagggaaagacctgcgggagcatctgaagagagaagggatgattttctcctgcccgc       c.*60

          .         .         .         .         .         .       g.151821
 cggtgtttgtgtgtttataatgcacaactcgcaaatataaattgcacatgcagaaggcac       c.*120

          .         .         .         .         .         .       g.151881
 agaccccgtagcgcatgccaacttgcagggactcggtttaatcttgtctcatgaatttcc       c.*180

          .         .         .         .         .         .       g.151941
 agatggcccactctcttccatatcacaaggacataaacactccttctttcagccccacct       c.*240

          .         .         .         .         .         .       g.152001
 ccccagggccctggaggagacccccaccctgcaatccacaccccatcctctgctgcagaa       c.*300

          .         .         .         .         .         .       g.152061
 gctatggtctgtgtggtgacagccagattctctactcttatgttttgtatttgttacata       c.*360

          .         .         .         .         .         .       g.152121
 ttctatttttataaagggaattttaaaaaataaatgtgttttgcacaaagctgtttatta       c.*420

                                                                    g.152130
 caaaaccca                                                          c.*429

 (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 Ellis van Creveld syndrome 2 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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