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

(used for variant description)

(last modified May 25, 2024)


This file was created to facilitate the description of sequence variants on transcript NM_001875.4 in the CPS1 gene based on a coding DNA reference sequence following the HGVS recommendations.

The sequence was taken from NC_000002.11, covering CPS1 transcript NM_001875.4.


Please note that introns are available by clicking on the exon numbers above the sequence.
 (upstream sequence)
                                                   .                g.83932
                                              aaagatcgctgtgca       c.-121

 .         .         .         .         .         .                g.83992
 gtcagccttaaacactgactgcacccctcccagatttcttttacattaactaaaaagtct       c.-61

 .         .         .         .         .         .                g.84052
 tatcacacaatctcataaaatttatgtaatttcatttaattttagccacaaatcatcaaa       c.-1

          .         .         .         .         .         .       g.84112
 ATGACGAGGATTTTGACAGCTTTCAAAGTGGTGAGGACACTGAAGACTGGTTTTGGCTTT       c.60
 M  T  R  I  L  T  A  F  K  V  V  R  T  L  K  T  G  F  G  F         p.20

          .         .         .         .         .         .       g.84172
 ACCAATGTGACTGCACACCAAAAATGGAAATTTTCAAGACCTGGCATCAGGCTCCTTTCT       c.120
 T  N  V  T  A  H  Q  K  W  K  F  S  R  P  G  I  R  L  L  S         p.40

        | 02 .         .         .         .         .         .    g.100670
 GTCAAG | GCACAGACAGCACACATTGTCCTGGAAGATGGAACTAAGATGAAAGGTTACTCC    c.180
 V  K   | A  Q  T  A  H  I  V  L  E  D  G  T  K  M  K  G  Y  S      p.60

          .         .         .         .         .       | 03 .    g.103668
 TTTGGCCATCCATCCTCTGTTGCTGGTGAAGTGGTTTTTAATACTGGCCTGGGAGG | GTAC    c.240
 F  G  H  P  S  S  V  A  G  E  V  V  F  N  T  G  L  G  G  |  Y      p.80

          .         .         .         .         .         .       g.103728
 CCAGAAGCTATTACTGACCCTGCCTACAAAGGACAGATTCTCACAATGGCCAACCCTATT       c.300
 P  E  A  I  T  D  P  A  Y  K  G  Q  I  L  T  M  A  N  P  I         p.100

          .         .         .         .         .         .       g.103788
 ATTGGGAATGGTGGAGCTCCTGATACTACTGCTCTGGATGAACTGGGACTTAGCAAATAT       c.360
 I  G  N  G  G  A  P  D  T  T  A  L  D  E  L  G  L  S  K  Y         p.120

          .         .  | 04      .         .         .         .    g.104778
 TTGGAGTCTAATGGAATCAAG | GTTTCAGGTTTGCTGGTGCTGGATTATAGTAAAGACTAC    c.420
 L  E  S  N  G  I  K   | V  S  G  L  L  V  L  D  Y  S  K  D  Y      p.140

          .         .         .         .         .  | 05      .    g.107041
 AACCACTGGCTGGCTACCAAGAGTTTAGGGCAATGGCTACAGGAAGAAAAG | GTTCCTGCA    c.480
 N  H  W  L  A  T  K  S  L  G  Q  W  L  Q  E  E  K   | V  P  A      p.160

          .         .         .         .         | 06         .    g.109947
 ATTTATGGAGTGGACACAAGAATGCTGACTAAAATAATTCGGGATAAG | GGTACCATGCTT    c.540
 I  Y  G  V  D  T  R  M  L  T  K  I  I  R  D  K   | G  T  M  L      p.180

          .         .         .         .         .         .       g.110007
 GGGAAGATTGAATTTGAAGGTCAGCCTGTGGATTTTGTGGATCCAAATAAACAGAATTTG       c.600
 G  K  I  E  F  E  G  Q  P  V  D  F  V  D  P  N  K  Q  N  L         p.200

          .         .  | 07      .         .         .         .    g.115415
 ATTGCTGAGGTTTCAACCAAG | GATGTCAAAGTGTACGGCAAAGGAAACCCCACAAAAGTG    c.660
 I  A  E  V  S  T  K   | D  V  K  V  Y  G  K  G  N  P  T  K  V      p.220

          .         .         .         .         .  | 08      .    g.117433
 GTAGCTGTAGACTGTGGGATTAAAAACAATGTAATCCGCCTGCTAGTAAAG | CGAGGAGCT    c.720
 V  A  V  D  C  G  I  K  N  N  V  I  R  L  L  V  K   | R  G  A      p.240

          .         .         .         .         .         .       g.117493
 GAAGTGCACTTAGTTCCCTGGAACCATGATTTCACCAAGATGGAGTATGATGGGATTTTG       c.780
 E  V  H  L  V  P  W  N  H  D  F  T  K  M  E  Y  D  G  I  L         p.260

          .         .         .         .         .         .       g.117553
 ATCGCGGGAGGACCGGGGAACCCAGCTCTTGCAGAACCACTAATTCAGAATGTCAGAAAG       c.840
 I  A  G  G  P  G  N  P  A  L  A  E  P  L  I  Q  N  V  R  K         p.280

  | 09       .         .         .         .         .         .    g.118178
  | ATTTTGGAGAGTGATCGCAAGGAGCCATTGTTTGGAATCAGTACAGGAAACTTAATAACA    c.900
  | I  L  E  S  D  R  K  E  P  L  F  G  I  S  T  G  N  L  I  T      p.300

          .         .         .         .        | 10.         .    g.119162
 GGATTGGCTGCTGGTGCCAAAACCTACAAGATGTCCATGGCCAACAG | AGGGCAGAATCAG    c.960
 G  L  A  A  G  A  K  T  Y  K  M  S  M  A  N  R  |  G  Q  N  Q      p.320

          .         .         .         .         .         .       g.119222
 CCTGTTTTGAATATCACAAACAAACAGGCTTTCATTACTGCTCAGAATCATGGCTATGCC       c.1020
 P  V  L  N  I  T  N  K  Q  A  F  I  T  A  Q  N  H  G  Y  A         p.340

          .         .         .         .         .         .       g.119282
 TTGGACAACACCCTCCCTGCTGGCTGGAAACCACTTTTTGTGAATGTCAACGATCAAACA       c.1080
 L  D  N  T  L  P  A  G  W  K  P  L  F  V  N  V  N  D  Q  T         p.360

        | 11 .         .         .         .         .         .    g.120251
 AATGAG | GGGATTATGCATGAGAGCAAACCCTTCTTCGCTGTGCAGTTCCACCCAGAGGTC    c.1140
 N  E   | G  I  M  H  E  S  K  P  F  F  A  V  Q  F  H  P  E  V      p.380

          .         .     | 12   .         .         .         .    g.121862
 ACCCCGGGGCCAATAGACACTGAG | TACCTGTTTGATTCCTTTTTCTCACTGATAAAGAAA    c.1200
 T  P  G  P  I  D  T  E   | Y  L  F  D  S  F  F  S  L  I  K  K      p.400

          .         .         .         .         .         .       g.121922
 GGAAAAGCTACCACCATTACATCAGTCTTACCGAAGCCAGCACTAGTTGCATCTCGGGTT       c.1260
 G  K  A  T  T  I  T  S  V  L  P  K  P  A  L  V  A  S  R  V         p.420

     | 13    .         .         .         .         .         .    g.122862
 GAG | GTTTCCAAAGTCCTTATTCTAGGATCAGGAGGTCTGTCCATTGGTCAGGCTGGAGAA    c.1320
 E   | V  S  K  V  L  I  L  G  S  G  G  L  S  I  G  Q  A  G  E      p.440

          .         .         .          | 14        .         .    g.126711
 TTTGATTACTCAGGATCTCAAGCTGTAAAAGCCATGAAG | GAAGAAAATGTCAAAACTGTT    c.1380
 F  D  Y  S  G  S  Q  A  V  K  A  M  K   | E  E  N  V  K  T  V      p.460

          .         .         .         .         .         .       g.126771
 CTGATGAACCCAAACATTGCATCAGTCCAGACCAATGAGGTGGGCTTAAAGCAAGCGGAT       c.1440
 L  M  N  P  N  I  A  S  V  Q  T  N  E  V  G  L  K  Q  A  D         p.480

          .         .         .         .         .         .       g.126831
 ACTGTCTACTTTCTTCCCATCACCCCTCAGTTTGTCACAGAGGTCATCAAGGCAGAACAG       c.1500
 T  V  Y  F  L  P  I  T  P  Q  F  V  T  E  V  I  K  A  E  Q         p.500

          .         .         .         .          | 15        .    g.127884
 CCAGATGGGTTAATTCTGGGCATGGGTGGCCAGACAGCTCTGAACTGTG | GAGTGGAACTA    c.1560
 P  D  G  L  I  L  G  M  G  G  Q  T  A  L  N  C  G |   V  E  L      p.520

          .         .         .         .         .         .       g.127944
 TTCAAGAGAGGTGTGCTCAAGGAATATGGTGTGAAAGTCCTGGGAACTTCAGTTGAGTCC       c.1620
 F  K  R  G  V  L  K  E  Y  G  V  K  V  L  G  T  S  V  E  S         p.540

          .         .         .         .         .         .       g.128004
 ATTATGGCTACGGAAGACAGGCAGCTGTTTTCAGATAAACTAAATGAGATCAATGAAAAG       c.1680
 I  M  A  T  E  D  R  Q  L  F  S  D  K  L  N  E  I  N  E  K         p.560

          .         .        | 16.         .         .         .    g.129553
 ATTGCTCCAAGTTTTGCAGTGGAATCG | ATTGAGGATGCACTGAAGGCAGCAGACACCATT    c.1740
 I  A  P  S  F  A  V  E  S   | I  E  D  A  L  K  A  A  D  T  I      p.580

          .         .         .         .         .         .       g.129613
 GGCTACCCAGTGATGATCCGTTCCGCCTATGCACTGGGTGGGTTAGGCTCAGGCATCTGT       c.1800
 G  Y  P  V  M  I  R  S  A  Y  A  L  G  G  L  G  S  G  I  C         p.600

          .         .         .       | 17 .         .         .    g.132444
 CCCAACAGAGAGACTTTGATGGACCTCAGCACAAAG | GCCTTTGCTATGACCAACCAAATT    c.1860
 P  N  R  E  T  L  M  D  L  S  T  K   | A  F  A  M  T  N  Q  I      p.620

          .         .         .         .         .         .       g.132504
 CTGGTGGAGAAGTCAGTGACAGGTTGGAAAGAAATAGAATATGAAGTGGTTCGAGATGCT       c.1920
 L  V  E  K  S  V  T  G  W  K  E  I  E  Y  E  V  V  R  D  A         p.640

          .         .         .         .         .         .       g.132564
 GATGACAATTGTGTCACTGTCTGTAACATGGAAAATGTTGATGCCATGGGTGTTCACACA       c.1980
 D  D  N  C  V  T  V  C  N  M  E  N  V  D  A  M  G  V  H  T         p.660

   | 18      .         .         .         .         .         .    g.134108
 G | GTGACTCAGTTGTTGTGGCTCCTGCCCAGACACTCTCCAATGCCGAGTTTCAGATGTTG    c.2040
 G |   D  S  V  V  V  A  P  A  Q  T  L  S  N  A  E  F  Q  M  L      p.680

          .         .         .         .         .         .       g.134168
 AGACGTACTTCAATCAATGTTGTTCGCCACTTGGGCATTGTGGGTGAATGCAACATTCAG       c.2100
 R  R  T  S  I  N  V  V  R  H  L  G  I  V  G  E  C  N  I  Q         p.700

          .         .         .         .         .         .       g.134228
 TTTGCCCTTCATCCTACCTCAATGGAATACTGCATCATTGAAGTGAATGCCAGACTGTCC       c.2160
 F  A  L  H  P  T  S  M  E  Y  C  I  I  E  V  N  A  R  L  S         p.720

          .         .         .   | 19     .         .         .    g.135707
 CGAAGCTCTGCTCTGGCCTCAAAAGCCACTGG | CTACCCATTGGCATTCATTGCTGCAAAG    c.2220
 R  S  S  A  L  A  S  K  A  T  G  |  Y  P  L  A  F  I  A  A  K      p.740

          .         .         .         .         .         .       g.135767
 ATTGCCCTAGGAATCCCACTTCCAGAAATTAAGAACGTCGTATCCGGGAAGACATCAGCC       c.2280
 I  A  L  G  I  P  L  P  E  I  K  N  V  V  S  G  K  T  S  A         p.760

          .         .         .         .         .         .       g.135827
 TGTTTTGAACCTAGCCTGGATTACATGGTCACCAAGATTCCCCGCTGGGATCTTGACCGT       c.2340
 C  F  E  P  S  L  D  Y  M  V  T  K  I  P  R  W  D  L  D  R         p.780

          .         .         .         .         .  | 20      .    g.139444
 TTTCATGGAACATCTAGCCGAATTGGTAGCTCTATGAAAAGTGTAGGAGAG | GTCATGGCT    c.2400
 F  H  G  T  S  S  R  I  G  S  S  M  K  S  V  G  E   | V  M  A      p.800

          .         .         .         .         .         .       g.139504
 ATTGGTCGTACCTTTGAGGAGAGTTTCCAGAAAGCTTTACGGATGTGCCACCCATCTATA       c.2460
 I  G  R  T  F  E  E  S  F  Q  K  A  L  R  M  C  H  P  S  I         p.820

          .         .         .         .         .         .       g.139564
 GAAGGTTTCACTCCCCGTCTCCCAATGAACAAAGAATGGCCATCTAATTTAGATCTTAGA       c.2520
 E  G  F  T  P  R  L  P  M  N  K  E  W  P  S  N  L  D  L  R         p.840

          .         .         .         .         | 21         .    g.143753
 AAAGAGTTGTCTGAACCAAGCAGCACGCGTATCTATGCCATTGCCAAG | GCCATTGATGAC    c.2580
 K  E  L  S  E  P  S  S  T  R  I  Y  A  I  A  K   | A  I  D  D      p.860

          .         .         .         .         .         .       g.143813
 AACATGTCCCTTGATGAGATTGAGAAGCTCACATACATTGACAAGTGGTTTTTGTATAAG       c.2640
 N  M  S  L  D  E  I  E  K  L  T  Y  I  D  K  W  F  L  Y  K         p.880

          .         .         .         .        | 22.         .    g.165033
 ATGCGTGATATTTTAAACATGGAAAAGACACTGAAAGGCCTCAACAG | TGAGTCCATGACA    c.2700
 M  R  D  I  L  N  M  E  K  T  L  K  G  L  N  S  |  E  S  M  T      p.900

          .         .         .         .         .         .       g.165093
 GAAGAAACCCTGAAAAGGGCAAAGGAGATTGGGTTCTCAGATAAGCAGATTTCAAAATGC       c.2760
 E  E  T  L  K  R  A  K  E  I  G  F  S  D  K  Q  I  S  K  C         p.920

          .         .         .         .         .         .       g.165153
 CTTGGGCTCACTGAGGCCCAGACAAGGGAGCTGAGGTTAAAGAAAAACATCCACCCTTGG       c.2820
 L  G  L  T  E  A  Q  T  R  E  L  R  L  K  K  N  I  H  P  W         p.940

           | 23        .         .         .         .         .    g.166519
 GTTAAACAG | ATTGATACACTGGCTGCAGAATACCCATCAGTAACAAACTATCTCTATGTT    c.2880
 V  K  Q   | I  D  T  L  A  A  E  Y  P  S  V  T  N  Y  L  Y  V      p.960

          .      | 24  .         .         .         .         .    g.167359
 ACCTACAATGGTCAG | GAGCATGATGTCAATTTTGATGACCATGGAATGATGGTGCTAGGC    c.2940
 T  Y  N  G  Q   | E  H  D  V  N  F  D  D  H  G  M  M  V  L  G      p.980

          .          | 25        .         .         .         .    g.169843
 TGTGGTCCATATCACATTG | GCAGCAGTGTGGAATTTGATTGGTGTGCTGTCTCTAGTATC    c.3000
 C  G  P  Y  H  I  G |   S  S  V  E  F  D  W  C  A  V  S  S  I      p.1000

          .         .         .         .         .         .       g.169903
 CGCACACTGCGTCAACTTGGCAAGAAGACGGTGGTGGTGAATTGCAATCCTGAGACTGTG       c.3060
 R  T  L  R  Q  L  G  K  K  T  V  V  V  N  C  N  P  E  T  V         p.1020

          .         .         .         .         .         .       g.169963
 AGCACAGACTTTGATGAGTGTGACAAACTGTACTTTGAAGAGTTGTCCTTGGAGAGAATC       c.3120
 S  T  D  F  D  E  C  D  K  L  Y  F  E  E  L  S  L  E  R  I         p.1040

          .         .  | 26      .         .         .         .    g.175220
 CTAGACATCTACCATCAGGAG | GCATGTGGTGGCTGCATCATATCAGTTGGAGGCCAGATT    c.3180
 L  D  I  Y  H  Q  E   | A  C  G  G  C  I  I  S  V  G  G  Q  I      p.1060

          .         .         .         .         .         .       g.175280
 CCAAACAACCTGGCAGTTCCTCTATACAAGAATGGTGTCAAGATCATGGGCACAAGCCCC       c.3240
 P  N  N  L  A  V  P  L  Y  K  N  G  V  K  I  M  G  T  S  P         p.1080

          .         .         .         .         .         .       g.175340
 CTGCAGATCGACAGGGCTGAGGATCGCTCCATCTTCTCAGCTGTCTTGGATGAGCTGAAG       c.3300
 L  Q  I  D  R  A  E  D  R  S  I  F  S  A  V  L  D  E  L  K         p.1100

          .         .         .       | 27 .         .         .    g.175815
 GTGGCTCAGGCACCTTGGAAAGCTGTTAATACTTTG | AATGAAGCACTGGAATTTGCAAAG    c.3360
 V  A  Q  A  P  W  K  A  V  N  T  L   | N  E  A  L  E  F  A  K      p.1120

          .         .         .         .     | 28   .         .    g.177697
 TCTGTGGACTACCCCTGCTTGTTGAGGCCTTCCTATGTTTTGAG | TGGGTCTGCTATGAAT    c.3420
 S  V  D  Y  P  C  L  L  R  P  S  Y  V  L  S  |  G  S  A  M  N      p.1140

          .         .         .         .         .         .       g.177757
 GTGGTATTCTCTGAGGATGAGATGAAAAAATTCCTAGAAGAGGCGACTAGAGTTTCTCAG       c.3480
 V  V  F  S  E  D  E  M  K  K  F  L  E  E  A  T  R  V  S  Q         p.1160

  | 29       .         .         .         .         .         .    g.181403
  | GAGCACCCAGTGGTGCTGACAAAATTTGTTGAAGGGGCCCGAGAAGTAGAAATGGACGCT    c.3540
  | E  H  P  V  V  L  T  K  F  V  E  G  A  R  E  V  E  M  D  A      p.1180

          .         | 30         .         .         .         .    g.183885
 GTTGGCAAAGATGGAAGG | GTTATCTCTCATGCCATCTCTGAACATGTTGAAGATGCAGGT    c.3600
 V  G  K  D  G  R   | V  I  S  H  A  I  S  E  H  V  E  D  A  G      p.1200

          .         .         .         .         .         .       g.183945
 GTCCACTCGGGAGATGCCACTCTGATGCTGCCCACACAAACCATCAGCCAAGGGGCCATT       c.3660
 V  H  S  G  D  A  T  L  M  L  P  T  Q  T  I  S  Q  G  A  I         p.1220

        | 31 .         .         .         .         .         .    g.185971
 GAAAAG | GTGAAGGATGCTACCCGGAAGATTGCAAAGGCTTTTGCCATCTCTGGTCCATTC    c.3720
 E  K   | V  K  D  A  T  R  K  I  A  K  A  F  A  I  S  G  P  F      p.1240

          .         .         .       | 32 .         .         .    g.187827
 AACGTCCAATTTCTTGTCAAAGGAAATGATGTCTTG | GTGATTGAGTGTAACTTGAGAGCT    c.3780
 N  V  Q  F  L  V  K  G  N  D  V  L   | V  I  E  C  N  L  R  A      p.1260

          .         .         .         .         .         .       g.187887
 TCTCGATCCTTCCCCTTTGTTTCCAAGACTCTTGGGGTTGACTTCATTGATGTGGCCACC       c.3840
 S  R  S  F  P  F  V  S  K  T  L  G  V  D  F  I  D  V  A  T         p.1280

          .         .         .         .         .         .       g.187947
 AAGGTGATGATTGGAGAGAATGTTGATGAGAAACATCTTCCAACATTGGACCATCCCATA       c.3900
 K  V  M  I  G  E  N  V  D  E  K  H  L  P  T  L  D  H  P  I         p.1300

          .         .        | 33.         .         .         .    g.190474
 ATTCCTGCTGACTATGTTGCAATTAAG | GCTCCCATGTTTTCCTGGCCCCGGTTGAGGGAT    c.3960
 I  P  A  D  Y  V  A  I  K   | A  P  M  F  S  W  P  R  L  R  D      p.1320

          .         .         .         .   | 34     .         .    g.195522
 GCTGACCCCATTCTGAGATGTGAGATGGCTTCCACTGGAGAG | GTGGCTTGCTTTGGTGAA    c.4020
 A  D  P  I  L  R  C  E  M  A  S  T  G  E   | V  A  C  F  G  E      p.1340

          .         .         .         .         .         .       g.195582
 GGTATTCATACAGCCTTCCTAAAGGCAATGCTTTCCACAGGATTTAAGATACCCCAGAAA       c.4080
 G  I  H  T  A  F  L  K  A  M  L  S  T  G  F  K  I  P  Q  K         p.1360

          .         .  | 35      .         .         .         .    g.202259
 GGCATCCTGATAGGCATCCAG | CAATCATTCCGGCCAAGATTCCTTGGTGTGGCTGAACAA    c.4140
 G  I  L  I  G  I  Q   | Q  S  F  R  P  R  F  L  G  V  A  E  Q      p.1380

          .         .  | 36      .         .         .         .    g.203085
 TTACACAATGAAGGTTTCAAG | CTGTTTGCCACGGAAGCCACATCAGACTGGCTCAACGCC    c.4200
 L  H  N  E  G  F  K   | L  F  A  T  E  A  T  S  D  W  L  N  A      p.1400

          .         .         .         .         .         .       g.203145
 AACAATGTCCCTGCCACCCCAGTGGCATGGCCGTCTCAAGAAGGACAGAATCCCAGCCTC       c.4260
 N  N  V  P  A  T  P  V  A  W  P  S  Q  E  G  Q  N  P  S  L         p.1420

          .     | 37   .         .         .         .         .    g.204371
 TCTTCCATCAGAAA | ATTGATTAGAGATGGCAGCATTGACCTAGTGATTAACCTTCCCAAC    c.4320
 S  S  I  R  K  |  L  I  R  D  G  S  I  D  L  V  I  N  L  P  N      p.1440

          .         .         .         .         .         .       g.204431
 AACAACACTAAATTTGTCCATGATAATTATGTGATTCGGAGGACAGCTGTTGATAGTGGA       c.4380
 N  N  T  K  F  V  H  D  N  Y  V  I  R  R  T  A  V  D  S  G         p.1460

          .         .     | 38   .         .         .         .    g.205241
 ATCCCTCTCCTCACTAATTTTCAG | GTGACCAAACTTTTTGCTGAAGCTGTGCAGAAATCT    c.4440
 I  P  L  L  T  N  F  Q   | V  T  K  L  F  A  E  A  V  Q  K  S      p.1480

          .         .         .         .         .         .       g.205301
 CGCAAGGTGGACTCCAAGAGTCTTTTCCACTACAGGCAGTACAGTGCTGGAAAAGCAGCA       c.4500
 R  K  V  D  S  K  S  L  F  H  Y  R  Q  Y  S  A  G  K  A  A         p.1500

                                                                    g.205304
 TAG                                                                c.4503
 X                                                                  p.1500

          .         .         .         .         .         .       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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