carbamoyl-phosphate synthase 1, mitochondrial (CPS1) - coding DNA reference sequence

(used for variant description)

(last modified July 31, 2017)


This file was created to facilitate the description of sequence variants on transcript NM_001122633.2 in the CPS1 gene based on a coding DNA reference sequence following the HGVS recommendations.

The sequence was taken from NG_008285.1, covering CPS1 transcript NM_001122633.2.


Please note that introns are available by clicking on the exon numbers above the sequence.
 (upstream sequence)
                                         .         .                g.5022
                                       gctcttttaaaatagttgcttt       c.-61

 .         .         .         .         .         .                g.5082
 cttaggaaatgtagttgctttcttaacctcatcaaattcatgaagatttagccgaggccc       c.-1

     | 02    .         .         .         .         .         .    g.84094
 ATG | CCACAAATCATCAAAATGACGAGGATTTTGACAGCTTTCAAAGTGGTGAGGACACTG    c.60
 M   | P  Q  I  I  K  M  T  R  I  L  T  A  F  K  V  V  R  T  L      p.20

          .         .         .         .         .         .       g.84154
 AAGACTGGTTTTGGCTTTACCAATGTGACTGCACACCAAAAATGGAAATTTTCAAGACCT       c.120
 K  T  G  F  G  F  T  N  V  T  A  H  Q  K  W  K  F  S  R  P         p.40

          .         .     | 03   .         .         .         .    g.100652
 GGCATCAGGCTCCTTTCTGTCAAG | GCACAGACAGCACACATTGTCCTGGAAGATGGAACT    c.180
 G  I  R  L  L  S  V  K   | A  Q  T  A  H  I  V  L  E  D  G  T      p.60

          .         .         .         .         .         .       g.100712
 AAGATGAAAGGTTACTCCTTTGGCCATCCATCCTCTGTTGCTGGTGAAGTGGTTTTTAAT       c.240
 K  M  K  G  Y  S  F  G  H  P  S  S  V  A  G  E  V  V  F  N         p.80

          .     | 04   .         .         .         .         .    g.103710
 ACTGGCCTGGGAGG | GTACCCAGAAGCTATTACTGACCCTGCCTACAAAGGACAGATTCTC    c.300
 T  G  L  G  G  |  Y  P  E  A  I  T  D  P  A  Y  K  G  Q  I  L      p.100

          .         .         .         .         .         .       g.103770
 ACAATGGCCAACCCTATTATTGGGAATGGTGGAGCTCCTGATACTACTGCTCTGGATGAA       c.360
 T  M  A  N  P  I  I  G  N  G  G  A  P  D  T  T  A  L  D  E         p.120

          .         .         .          | 05        .         .    g.104760
 CTGGGACTTAGCAAATATTTGGAGTCTAATGGAATCAAG | GTTTCAGGTTTGCTGGTGCTG    c.420
 L  G  L  S  K  Y  L  E  S  N  G  I  K   | V  S  G  L  L  V  L      p.140

          .         .         .         .         .         .       g.104820
 GATTATAGTAAAGACTACAACCACTGGCTGGCTACCAAGAGTTTAGGGCAATGGCTACAG       c.480
 D  Y  S  K  D  Y  N  H  W  L  A  T  K  S  L  G  Q  W  L  Q         p.160

           | 06        .         .         .         .         .    g.107083
 GAAGAAAAG | GTTCCTGCAATTTATGGAGTGGACACAAGAATGCTGACTAAAATAATTCGG    c.540
 E  E  K   | V  P  A  I  Y  G  V  D  T  R  M  L  T  K  I  I  R      p.180

        | 07 .         .         .         .         .         .    g.109989
 GATAAG | GGTACCATGCTTGGGAAGATTGAATTTGAAGGTCAGCCTGTGGATTTTGTGGAT    c.600
 D  K   | G  T  M  L  G  K  I  E  F  E  G  Q  P  V  D  F  V  D      p.200

          .         .         .          | 08        .         .    g.115397
 CCAAATAAACAGAATTTGATTGCTGAGGTTTCAACCAAG | GATGTCAAAGTGTACGGCAAA    c.660
 P  N  K  Q  N  L  I  A  E  V  S  T  K   | D  V  K  V  Y  G  K      p.220

          .         .         .         .         .         .       g.115457
 GGAAACCCCACAAAAGTGGTAGCTGTAGACTGTGGGATTAAAAACAATGTAATCCGCCTG       c.720
 G  N  P  T  K  V  V  A  V  D  C  G  I  K  N  N  V  I  R  L         p.240

           | 09        .         .         .         .         .    g.117475
 CTAGTAAAG | CGAGGAGCTGAAGTGCACTTAGTTCCCTGGAACCATGATTTCACCAAGATG    c.780
 L  V  K   | R  G  A  E  V  H  L  V  P  W  N  H  D  F  T  K  M      p.260

          .         .         .         .         .         .       g.117535
 GAGTATGATGGGATTTTGATCGCGGGAGGACCGGGGAACCCAGCTCTTGCAGAACCACTA       c.840
 E  Y  D  G  I  L  I  A  G  G  P  G  N  P  A  L  A  E  P  L         p.280

          .         | 10         .         .         .         .    g.118160
 ATTCAGAATGTCAGAAAG | ATTTTGGAGAGTGATCGCAAGGAGCCATTGTTTGGAATCAGT    c.900
 I  Q  N  V  R  K   | I  L  E  S  D  R  K  E  P  L  F  G  I  S      p.300

          .         .         .         .         .         .       g.118220
 ACAGGAAACTTAATAACAGGATTGGCTGCTGGTGCCAAAACCTACAAGATGTCCATGGCC       c.960
 T  G  N  L  I  T  G  L  A  A  G  A  K  T  Y  K  M  S  M  A         p.320

       | 11  .         .         .         .         .         .    g.119204
 AACAG | AGGGCAGAATCAGCCTGTTTTGAATATCACAAACAAACAGGCTTTCATTACTGCT    c.1020
 N  R  |  G  Q  N  Q  P  V  L  N  I  T  N  K  Q  A  F  I  T  A      p.340

          .         .         .         .         .         .       g.119264
 CAGAATCATGGCTATGCCTTGGACAACACCCTCCCTGCTGGCTGGAAACCACTTTTTGTG       c.1080
 Q  N  H  G  Y  A  L  D  N  T  L  P  A  G  W  K  P  L  F  V         p.360

          .         .     | 12   .         .         .         .    g.120233
 AATGTCAACGATCAAACAAATGAG | GGGATTATGCATGAGAGCAAACCCTTCTTCGCTGTG    c.1140
 N  V  N  D  Q  T  N  E   | G  I  M  H  E  S  K  P  F  F  A  V      p.380

          .         .         .         .   | 13     .         .    g.121844
 CAGTTCCACCCAGAGGTCACCCCGGGGCCAATAGACACTGAG | TACCTGTTTGATTCCTTT    c.1200
 Q  F  H  P  E  V  T  P  G  P  I  D  T  E   | Y  L  F  D  S  F      p.400

          .         .         .         .         .         .       g.121904
 TTCTCACTGATAAAGAAAGGAAAAGCTACCACCATTACATCAGTCTTACCGAAGCCAGCA       c.1260
 F  S  L  I  K  K  G  K  A  T  T  I  T  S  V  L  P  K  P  A         p.420

          .         .  | 14      .         .         .         .    g.122844
 CTAGTTGCATCTCGGGTTGAG | GTTTCCAAAGTCCTTATTCTAGGATCAGGAGGTCTGTCC    c.1320
 L  V  A  S  R  V  E   | V  S  K  V  L  I  L  G  S  G  G  L  S      p.440

          .         .         .         .         .        | 15.    g.126693
 ATTGGTCAGGCTGGAGAATTTGATTACTCAGGATCTCAAGCTGTAAAAGCCATGAAG | GAA    c.1380
 I  G  Q  A  G  E  F  D  Y  S  G  S  Q  A  V  K  A  M  K   | E      p.460

          .         .         .         .         .         .       g.126753
 GAAAATGTCAAAACTGTTCTGATGAACCCAAACATTGCATCAGTCCAGACCAATGAGGTG       c.1440
 E  N  V  K  T  V  L  M  N  P  N  I  A  S  V  Q  T  N  E  V         p.480

          .         .         .         .         .         .       g.126813
 GGCTTAAAGCAAGCGGATACTGTCTACTTTCTTCCCATCACCCCTCAGTTTGTCACAGAG       c.1500
 G  L  K  Q  A  D  T  V  Y  F  L  P  I  T  P  Q  F  V  T  E         p.500

          .         .         .         .         .         .       g.126873
 GTCATCAAGGCAGAACAGCCAGATGGGTTAATTCTGGGCATGGGTGGCCAGACAGCTCTG       c.1560
 V  I  K  A  E  Q  P  D  G  L  I  L  G  M  G  G  Q  T  A  L         p.520

         | 16.         .         .         .         .         .    g.127926
 AACTGTG | GAGTGGAACTATTCAAGAGAGGTGTGCTCAAGGAATATGGTGTGAAAGTCCTG    c.1620
 N  C  G |   V  E  L  F  K  R  G  V  L  K  E  Y  G  V  K  V  L      p.540

          .         .         .         .         .         .       g.127986
 GGAACTTCAGTTGAGTCCATTATGGCTACGGAAGACAGGCAGCTGTTTTCAGATAAACTA       c.1680
 G  T  S  V  E  S  I  M  A  T  E  D  R  Q  L  F  S  D  K  L         p.560

          .         .         .         .      | 17  .         .    g.129535
 AATGAGATCAATGAAAAGATTGCTCCAAGTTTTGCAGTGGAATCG | ATTGAGGATGCACTG    c.1740
 N  E  I  N  E  K  I  A  P  S  F  A  V  E  S   | I  E  D  A  L      p.580

          .         .         .         .         .         .       g.129595
 AAGGCAGCAGACACCATTGGCTACCCAGTGATGATCCGTTCCGCCTATGCACTGGGTGGG       c.1800
 K  A  A  D  T  I  G  Y  P  V  M  I  R  S  A  Y  A  L  G  G         p.600

          .         .         .         .         .     | 18   .    g.132426
 TTAGGCTCAGGCATCTGTCCCAACAGAGAGACTTTGATGGACCTCAGCACAAAG | GCCTTT    c.1860
 L  G  S  G  I  C  P  N  R  E  T  L  M  D  L  S  T  K   | A  F      p.620

          .         .         .         .         .         .       g.132486
 GCTATGACCAACCAAATTCTGGTGGAGAAGTCAGTGACAGGTTGGAAAGAAATAGAATAT       c.1920
 A  M  T  N  Q  I  L  V  E  K  S  V  T  G  W  K  E  I  E  Y         p.640

          .         .         .         .         .         .       g.132546
 GAAGTGGTTCGAGATGCTGATGACAATTGTGTCACTGTCTGTAACATGGAAAATGTTGAT       c.1980
 E  V  V  R  D  A  D  D  N  C  V  T  V  C  N  M  E  N  V  D         p.660

          .          | 19        .         .         .         .    g.134090
 GCCATGGGTGTTCACACAG | GTGACTCAGTTGTTGTGGCTCCTGCCCAGACACTCTCCAAT    c.2040
 A  M  G  V  H  T  G |   D  S  V  V  V  A  P  A  Q  T  L  S  N      p.680

          .         .         .         .         .         .       g.134150
 GCCGAGTTTCAGATGTTGAGACGTACTTCAATCAATGTTGTTCGCCACTTGGGCATTGTG       c.2100
 A  E  F  Q  M  L  R  R  T  S  I  N  V  V  R  H  L  G  I  V         p.700

          .         .         .         .         .         .       g.134210
 GGTGAATGCAACATTCAGTTTGCCCTTCATCCTACCTCAATGGAATACTGCATCATTGAA       c.2160
 G  E  C  N  I  Q  F  A  L  H  P  T  S  M  E  Y  C  I  I  E         p.720

          .         .         .         .         . | 20       .    g.135689
 GTGAATGCCAGACTGTCCCGAAGCTCTGCTCTGGCCTCAAAAGCCACTGG | CTACCCATTG    c.2220
 V  N  A  R  L  S  R  S  S  A  L  A  S  K  A  T  G  |  Y  P  L      p.740

          .         .         .         .         .         .       g.135749
 GCATTCATTGCTGCAAAGATTGCCCTAGGAATCCCACTTCCAGAAATTAAGAACGTCGTA       c.2280
 A  F  I  A  A  K  I  A  L  G  I  P  L  P  E  I  K  N  V  V         p.760

          .         .         .         .         .         .       g.135809
 TCCGGGAAGACATCAGCCTGTTTTGAACCTAGCCTGGATTACATGGTCACCAAGATTCCC       c.2340
 S  G  K  T  S  A  C  F  E  P  S  L  D  Y  M  V  T  K  I  P         p.780

          .         .         .         .         .         .       g.135869
 CGCTGGGATCTTGACCGTTTTCATGGAACATCTAGCCGAATTGGTAGCTCTATGAAAAGT       c.2400
 R  W  D  L  D  R  F  H  G  T  S  S  R  I  G  S  S  M  K  S         p.800

           | 21        .         .         .         .         .    g.139486
 GTAGGAGAG | GTCATGGCTATTGGTCGTACCTTTGAGGAGAGTTTCCAGAAAGCTTTACGG    c.2460
 V  G  E   | V  M  A  I  G  R  T  F  E  E  S  F  Q  K  A  L  R      p.820

          .         .         .         .         .         .       g.139546
 ATGTGCCACCCATCTATAGAAGGTTTCACTCCCCGTCTCCCAATGAACAAAGAATGGCCA       c.2520
 M  C  H  P  S  I  E  G  F  T  P  R  L  P  M  N  K  E  W  P         p.840

          .         .         .         .         .         .       g.139606
 TCTAATTTAGATCTTAGAAAAGAGTTGTCTGAACCAAGCAGCACGCGTATCTATGCCATT       c.2580
 S  N  L  D  L  R  K  E  L  S  E  P  S  S  T  R  I  Y  A  I         p.860

        | 22 .         .         .         .         .         .    g.143795
 GCCAAG | GCCATTGATGACAACATGTCCCTTGATGAGATTGAGAAGCTCACATACATTGAC    c.2640
 A  K   | A  I  D  D  N  M  S  L  D  E  I  E  K  L  T  Y  I  D      p.880

          .         .         .         .         .         .       g.143855
 AAGTGGTTTTTGTATAAGATGCGTGATATTTTAAACATGGAAAAGACACTGAAAGGCCTC       c.2700
 K  W  F  L  Y  K  M  R  D  I  L  N  M  E  K  T  L  K  G  L         p.900

       | 23  .         .         .         .         .         .    g.165075
 AACAG | TGAGTCCATGACAGAAGAAACCCTGAAAAGGGCAAAGGAGATTGGGTTCTCAGAT    c.2760
 N  S  |  E  S  M  T  E  E  T  L  K  R  A  K  E  I  G  F  S  D      p.920

          .         .         .         .         .         .       g.165135
 AAGCAGATTTCAAAATGCCTTGGGCTCACTGAGGCCCAGACAAGGGAGCTGAGGTTAAAG       c.2820
 K  Q  I  S  K  C  L  G  L  T  E  A  Q  T  R  E  L  R  L  K         p.940

          .         .        | 24.         .         .         .    g.166501
 AAAAACATCCACCCTTGGGTTAAACAG | ATTGATACACTGGCTGCAGAATACCCATCAGTA    c.2880
 K  N  I  H  P  W  V  K  Q   | I  D  T  L  A  A  E  Y  P  S  V      p.960

          .         .         .    | 25    .         .         .    g.167341
 ACAAACTATCTCTATGTTACCTACAATGGTCAG | GAGCATGATGTCAATTTTGATGACCAT    c.2940
 T  N  Y  L  Y  V  T  Y  N  G  Q   | E  H  D  V  N  F  D  D  H      p.980

          .         .         .        | 26.         .         .    g.169825
 GGAATGATGGTGCTAGGCTGTGGTCCATATCACATTG | GCAGCAGTGTGGAATTTGATTGG    c.3000
 G  M  M  V  L  G  C  G  P  Y  H  I  G |   S  S  V  E  F  D  W      p.1000

          .         .         .         .         .         .       g.169885
 TGTGCTGTCTCTAGTATCCGCACACTGCGTCAACTTGGCAAGAAGACGGTGGTGGTGAAT       c.3060
 C  A  V  S  S  I  R  T  L  R  Q  L  G  K  K  T  V  V  V  N         p.1020

          .         .         .         .         .         .       g.169945
 TGCAATCCTGAGACTGTGAGCACAGACTTTGATGAGTGTGACAAACTGTACTTTGAAGAG       c.3120
 C  N  P  E  T  V  S  T  D  F  D  E  C  D  K  L  Y  F  E  E         p.1040

          .         .         .          | 27        .         .    g.175202
 TTGTCCTTGGAGAGAATCCTAGACATCTACCATCAGGAG | GCATGTGGTGGCTGCATCATA    c.3180
 L  S  L  E  R  I  L  D  I  Y  H  Q  E   | A  C  G  G  C  I  I      p.1060

          .         .         .         .         .         .       g.175262
 TCAGTTGGAGGCCAGATTCCAAACAACCTGGCAGTTCCTCTATACAAGAATGGTGTCAAG       c.3240
 S  V  G  G  Q  I  P  N  N  L  A  V  P  L  Y  K  N  G  V  K         p.1080

          .         .         .         .         .         .       g.175322
 ATCATGGGCACAAGCCCCCTGCAGATCGACAGGGCTGAGGATCGCTCCATCTTCTCAGCT       c.3300
 I  M  G  T  S  P  L  Q  I  D  R  A  E  D  R  S  I  F  S  A         p.1100

          .         .         .         .         .     | 28   .    g.175797
 GTCTTGGATGAGCTGAAGGTGGCTCAGGCACCTTGGAAAGCTGTTAATACTTTG | AATGAA    c.3360
 V  L  D  E  L  K  V  A  Q  A  P  W  K  A  V  N  T  L   | N  E      p.1120

          .         .         .         .         .         .       g.175857
 GCACTGGAATTTGCAAAGTCTGTGGACTACCCCTGCTTGTTGAGGCCTTCCTATGTTTTG       c.3420
 A  L  E  F  A  K  S  V  D  Y  P  C  L  L  R  P  S  Y  V  L         p.1140

    | 29     .         .         .         .         .         .    g.177739
 AG | TGGGTCTGCTATGAATGTGGTATTCTCTGAGGATGAGATGAAAAAATTCCTAGAAGAG    c.3480
 S  |  G  S  A  M  N  V  V  F  S  E  D  E  M  K  K  F  L  E  E      p.1160

          .         | 30         .         .         .         .    g.181385
 GCGACTAGAGTTTCTCAG | GAGCACCCAGTGGTGCTGACAAAATTTGTTGAAGGGGCCCGA    c.3540
 A  T  R  V  S  Q   | E  H  P  V  V  L  T  K  F  V  E  G  A  R      p.1180

          .         .         .       | 31 .         .         .    g.183867
 GAAGTAGAAATGGACGCTGTTGGCAAAGATGGAAGG | GTTATCTCTCATGCCATCTCTGAA    c.3600
 E  V  E  M  D  A  V  G  K  D  G  R   | V  I  S  H  A  I  S  E      p.1200

          .         .         .         .         .         .       g.183927
 CATGTTGAAGATGCAGGTGTCCACTCGGGAGATGCCACTCTGATGCTGCCCACACAAACC       c.3660
 H  V  E  D  A  G  V  H  S  G  D  A  T  L  M  L  P  T  Q  T         p.1220

          .         .     | 32   .         .         .         .    g.185953
 ATCAGCCAAGGGGCCATTGAAAAG | GTGAAGGATGCTACCCGGAAGATTGCAAAGGCTTTT    c.3720
 I  S  Q  G  A  I  E  K   | V  K  D  A  T  R  K  I  A  K  A  F      p.1240

          .         .         .         .         .     | 33   .    g.187809
 GCCATCTCTGGTCCATTCAACGTCCAATTTCTTGTCAAAGGAAATGATGTCTTG | GTGATT    c.3780
 A  I  S  G  P  F  N  V  Q  F  L  V  K  G  N  D  V  L   | V  I      p.1260

          .         .         .         .         .         .       g.187869
 GAGTGTAACTTGAGAGCTTCTCGATCCTTCCCCTTTGTTTCCAAGACTCTTGGGGTTGAC       c.3840
 E  C  N  L  R  A  S  R  S  F  P  F  V  S  K  T  L  G  V  D         p.1280

          .         .         .         .         .         .       g.187929
 TTCATTGATGTGGCCACCAAGGTGATGATTGGAGAGAATGTTGATGAGAAACATCTTCCA       c.3900
 F  I  D  V  A  T  K  V  M  I  G  E  N  V  D  E  K  H  L  P         p.1300

          .         .         .         .      | 34  .         .    g.190456
 ACATTGGACCATCCCATAATTCCTGCTGACTATGTTGCAATTAAG | GCTCCCATGTTTTCC    c.3960
 T  L  D  H  P  I  I  P  A  D  Y  V  A  I  K   | A  P  M  F  S      p.1320

          .         .         .         .         .         .       g.190516
 TGGCCCCGGTTGAGGGATGCTGACCCCATTCTGAGATGTGAGATGGCTTCCACTGGAGAG       c.4020
 W  P  R  L  R  D  A  D  P  I  L  R  C  E  M  A  S  T  G  E         p.1340

  | 35       .         .         .         .         .         .    g.195564
  | GTGGCTTGCTTTGGTGAAGGTATTCATACAGCCTTCCTAAAGGCAATGCTTTCCACAGGA    c.4080
  | V  A  C  F  G  E  G  I  H  T  A  F  L  K  A  M  L  S  T  G      p.1360

          .         .         .          | 36        .         .    g.202241
 TTTAAGATACCCCAGAAAGGCATCCTGATAGGCATCCAG | CAATCATTCCGGCCAAGATTC    c.4140
 F  K  I  P  Q  K  G  I  L  I  G  I  Q   | Q  S  F  R  P  R  F      p.1380

          .         .         .          | 37        .         .    g.203067
 CTTGGTGTGGCTGAACAATTACACAATGAAGGTTTCAAG | CTGTTTGCCACGGAAGCCACA    c.4200
 L  G  V  A  E  Q  L  H  N  E  G  F  K   | L  F  A  T  E  A  T      p.1400

          .         .         .         .         .         .       g.203127
 TCAGACTGGCTCAACGCCAACAATGTCCCTGCCACCCCAGTGGCATGGCCGTCTCAAGAA       c.4260
 S  D  W  L  N  A  N  N  V  P  A  T  P  V  A  W  P  S  Q  E         p.1420

          .         .         .   | 38     .         .         .    g.204353
 GGACAGAATCCCAGCCTCTCTTCCATCAGAAA | ATTGATTAGAGATGGCAGCATTGACCTA    c.4320
 G  Q  N  P  S  L  S  S  I  R  K  |  L  I  R  D  G  S  I  D  L      p.1440

          .         .         .         .         .         .       g.204413
 GTGATTAACCTTCCCAACAACAACACTAAATTTGTCCATGATAATTATGTGATTCGGAGG       c.4380
 V  I  N  L  P  N  N  N  T  K  F  V  H  D  N  Y  V  I  R  R         p.1460

          .         .         .         .   | 39     .         .    g.205223
 ACAGCTGTTGATAGTGGAATCCCTCTCCTCACTAATTTTCAG | GTGACCAAACTTTTTGCT    c.4440
 T  A  V  D  S  G  I  P  L  L  T  N  F  Q   | V  T  K  L  F  A      p.1480

          .         .         .         .         .         .       g.205283
 GAAGCTGTGCAGAAATCTCGCAAGGTGGACTCCAAGAGTCTTTTCCACTACAGGCAGTAC       c.4500
 E  A  V  Q  K  S  R  K  V  D  S  K  S  L  F  H  Y  R  Q  Y         p.1500

          .         .                                               g.205304
 AGTGCTGGAAAAGCAGCATAG                                              c.4521
 S  A  G  K  A  A  X                                                p.1506

          .         .         .         .         .         .       g.205364
 agatgcagacaccccagccccattattaaatcaacctgagccacatgttatctaaaggaa       c.*60

          .         .         .         .         .         .       g.205424
 ctgattcacaactttctcagagatgaatattgataactaaacttcatttcagtttacttt       c.*120

          .         .         .         .         .         .       g.205484
 gttatgccttaatattctgtgtcttttgcaattaaattgtcagtcacttcttcaaaacct       c.*180

          .         .         .         .         .         .       g.205544
 tacagtccttcctaagttactcttcatgagatttcatccatttactaatactgtattttt       c.*240

          .         .         .         .         .         .       g.205604
 ggtggactaggcttgcctatgtgcttatgtgtagctttttactttttatggtgctgatta       c.*300

          .         .         .         .         .         .       g.205664
 atggtgatcaaggtaggaaaagttgctgttctattttctgaactctttctatactttaag       c.*360

          .         .         .         .         .         .       g.205724
 atactctatttttaaaacactatctgcaaactcaggacactttaacagggcagaatactc       c.*420

          .         .         .         .         .         .       g.205784
 taaaaacttgataaaattaaatatagatttaatttatgaaccttccatcatgatgtttgt       c.*480

          .         .         .         .         .         .       g.205844
 gtattgcttctttttggatcctcattctcacccatttggctaatccaggaatattgttat       c.*540

          .         .         .         .         .         .       g.205904
 cccttcccattatattgaagttgagaaatgtgacagaggcatttagagtatggacttttc       c.*600

          .         .         .         .         .         .       g.205964
 ttttctttttctttttctttttttctttttgagatggagtcacactctccaggctggagt       c.*660

          .         .         .         .         .         .       g.206024
 gcagtggcacaatctcggctcactgcaatttccgtctcccaagttcaagcgattctcctg       c.*720

          .         .         .         .         .         .       g.206084
 ctttagactatggatttctttaaggaatactggtttgcagttttgttttctggactatat       c.*780

          .         .         .         .         .         .       g.206144
 cagcagatggtagacagtgtttatgtagatgtgttgttgtttttatcattggattttaac       c.*840

          .         .         .         .         .         .       g.206204
 ttggcccgagtgaaataatcagatttttgtcattcacactctcccccagttttggaataa       c.*900

          .         .         .         .         .         .       g.206264
 cttggaagtaaggttcattcccttaagacgatggattctgttgaactatggggtcccaca       c.*960

          .         .         .         .         .         .       g.206324
 ctgcactattaattccacccactgtaagggcaaggacaccattccttctacatataagaa       c.*1020

          .         .         .         .         .         .       g.206384
 aaaagtctctccccaagggcagcctttgttacttttaaatattttctgttattacaagtg       c.*1080

          .         .         .         .                           g.206426
 ctctaattgtgaacttttaaataaaatactattaagaggtaa                         c.*1122

 (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 Carbamoyl-phosphate synthase 1, mitochondrial 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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