methylcrotonoyl-CoA carboxylase 1 (alpha) (MCCC1) - coding DNA reference sequence

(used for variant description)

(last modified July 12, 2024)


This file was created to facilitate the description of sequence variants on transcript NM_020166.3 in the MCCC1 gene based on a coding DNA reference sequence following the HGVS recommendations.

The sequence was taken from NC_000003.11, covering MCCC1 transcript NM_020166.3.


Please note that introns are available by clicking on the exon numbers above the sequence.
 (upstream sequence)
                                                   .                g.5017
                                            atcagcccaaaggtagg       c.-121

 .         .         .         .         .         .                g.5077
 ctcaggctccgacggtggccggcgggggtcacgaggcttcgtagtggaggaacgggtttg       c.-61

 .         .         .         .         .         .                g.5137
 gcgtgtgggacgcagctgcctctgtactggggagtcacggagtggccgggctccagggac       c.-1

          .         .         .         .         .         .       g.5197
 ATGGCGGCGGCCTCTGCGGTGTCGGTGCTGCTGGTGGCGGCGGAGAGGAACCGGTGGCAT       c.60
 M  A  A  A  S  A  V  S  V  L  L  V  A  A  E  R  N  R  W  H         p.20

          .         .          | 02        .         .         .    g.10003
 CGTCTCCCGAGCCTGCTCCTGCCGCCGAG | GACATGGGTGTGGAGGCAAAGAACCATGAAG    c.120
 R  L  P  S  L  L  L  P  P  R  |  T  W  V  W  R  Q  R  T  M  K      p.40

          .       | 03 .         .         .         .         .    g.12076
 TACACAACAGCCACAG | GAAGAAACATTACCAAGGTCCTCATTGCAAACAGAGGAGAAATT    c.180
 Y  T  T  A  T  G |   R  N  I  T  K  V  L  I  A  N  R  G  E  I      p.60

          .         .         .         .         .         .       g.12136
 GCCTGCAGGGTGATGCGCACAGCCAAAAAACTGGGTGTACAGACTGTGGCGGTTTATAGT       c.240
 A  C  R  V  M  R  T  A  K  K  L  G  V  Q  T  V  A  V  Y  S         p.80

          .         .         .    | 04    .         .         .    g.17816
 GAGGCTGACAGAAATTCCATGCATGTAGATATG | GCAGATGAAGCATATTCCATCGGCCCC    c.300
 E  A  D  R  N  S  M  H  V  D  M   | A  D  E  A  Y  S  I  G  P      p.100

          .         .         .         .         .         .       g.17876
 GCTCCCTCCCAGCAGAGCTACCTATCTATGGAGAAAATCATTCAAGTGGCCAAGACCTCT       c.360
 A  P  S  Q  Q  S  Y  L  S  M  E  K  I  I  Q  V  A  K  T  S         p.120

           | 05        .         .         .         .         .    g.32141
 GCTGCACAG | GCTATCCATCCAGGATGCGGTTTTCTCTCAGAAAACATGGAATTTGCTGAA    c.420
 A  A  Q   | A  I  H  P  G  C  G  F  L  S  E  N  M  E  F  A  E      p.140

          .         .         .         .         .         .       g.32201
 CTTTGTAAGCAAGAAGGAATTATTTTTATAGGCCCTCCTCCATCTGCAATTAGAGACATG       c.480
 L  C  K  Q  E  G  I  I  F  I  G  P  P  P  S  A  I  R  D  M         p.160

          .  | 06      .         .         .         .         .    g.33269
 GGTATAAAGAG | CACATCCAAATCCATAATGGCTGCTGCTGGAGTACCTGTTGTGGAGGGT    c.540
 G  I  K  S  |  T  S  K  S  I  M  A  A  A  G  V  P  V  V  E  G      p.180

          .         .         .         .         .         .       g.33329
 TATCATGGTGAGGACCAATCAGACCAGTGCCTGAAGGAACACGCCAGGAGAATTGGCTAT       c.600
 Y  H  G  E  D  Q  S  D  Q  C  L  K  E  H  A  R  R  I  G  Y         p.200

          .         .         .          | 07        .         .    g.33478
 CCTGTCATGATTAAAGCCGTCCGGGGTGGAGGAGGAAAA | GGAATGAGGATTGTTAGATCA    c.660
 P  V  M  I  K  A  V  R  G  G  G  G  K   | G  M  R  I  V  R  S      p.220

          .         .         .         .         .         .       g.33538
 GAACAAGAATTTCAAGAACAGTTAGAGTCAGCACGGAGAGAAGCTAAGAAGTCTTTCAAT       c.720
 E  Q  E  F  Q  E  Q  L  E  S  A  R  R  E  A  K  K  S  F  N         p.240

          .         .         .         .  | 08      .         .    g.47174
 GATGATGCTATGCTGATCGAGAAGTTTGTAGACACACCGAG | GCATGTAGAAGTCCAGGTG    c.780
 D  D  A  M  L  I  E  K  F  V  D  T  P  R  |  H  V  E  V  Q  V      p.260

          .         .         .         .         .         .       g.47234
 TTTGGTGATCACCATGGCAATGCTGTGTACTTGTTTGAAAGAGACTGTAGTGTGCAGAGG       c.840
 F  G  D  H  H  G  N  A  V  Y  L  F  E  R  D  C  S  V  Q  R         p.280

          .         .         .    | 09    .         .         .    g.52364
 CGACATCAGAAGATCATTGAGGAGGCCCCAGCG | CCTGGTATTAAATCTGAAGTAAGAAAA    c.900
 R  H  Q  K  I  I  E  E  A  P  A   | P  G  I  K  S  E  V  R  K      p.300

          .         .         .         .         .      | 10  .    g.59042
 AAGCTGGGAGAAGCTGCAGTCAGAGCTGCTAAAGCTGTAAATTATGTTGGAGCAG | GGACT    c.960
 K  L  G  E  A  A  V  R  A  A  K  A  V  N  Y  V  G  A  G |   T      p.320

          .         .         .         .         .         .       g.59102
 GTGGAGTTTATTATGGACTCAAAACATAATTTCTGTTTCATGGAGATGAATACAAGGCTG       c.1020
 V  E  F  I  M  D  S  K  H  N  F  C  F  M  E  M  N  T  R  L         p.340

          .         .         .         .         .         .       g.59162
 CAAGTGGAACATCCTGTTACTGAGATGATCACAGGAACTGACTTGGTGGAGTGGCAGCTT       c.1080
 Q  V  E  H  P  V  T  E  M  I  T  G  T  D  L  V  E  W  Q  L         p.360

     | 11    .         .         .         .         .         .    g.62884
 AGA | ATTGCAGCAGGAGAGAAGATTCCTTTGAGCCAGGAAGAAATAACTCTGCAGGGCCAT    c.1140
 R   | I  A  A  G  E  K  I  P  L  S  Q  E  E  I  T  L  Q  G  H      p.380

          .         .         .         .         .         .       g.62944
 GCCTTCGAAGCTAGAATATATGCAGAAGATCCTAGCAATAACTTCATGCCTGTGGCAGGC       c.1200
 A  F  E  A  R  I  Y  A  E  D  P  S  N  N  F  M  P  V  A  G         p.400

          .         .         .         .         .         .       g.63004
 CCATTAGTGCACCTCTCTACTCCTCGAGCAGACCCTTCCACCAGGATTGAAACTGGAGTA       c.1260
 P  L  V  H  L  S  T  P  R  A  D  P  S  T  R  I  E  T  G  V         p.420

         | 12.         .         .         .         .         .    g.65495
 CGGCAAG | GAGACGAAGTTTCCGTGCATTATGACCCCATGATTGCGAAGCTGGTCGTGTGG    c.1320
 R  Q  G |   D  E  V  S  V  H  Y  D  P  M  I  A  K  L  V  V  W      p.440

          .         .         .         .         .        | 13.    g.67146
 GCAGCAGATCGCCAGGCGGCATTGACAAAACTGAGGTACAGCCTTCGTCAGTACAAT | ATT    c.1380
 A  A  D  R  Q  A  A  L  T  K  L  R  Y  S  L  R  Q  Y  N   | I      p.460

          .         .         .         .         .         .       g.67206
 GTTGGACTGCACACCAACATTGACTTCTTACTCAACCTGTCTGGCCACCCAGAGTTTGAA       c.1440
 V  G  L  H  T  N  I  D  F  L  L  N  L  S  G  H  P  E  F  E         p.480

          .         .         .         .         .         .       g.67266
 GCTGGGAACGTGCACACTGATTTCATCCCTCAACACCACAAACAGTTGTTGCTCAGTCGG       c.1500
 A  G  N  V  H  T  D  F  I  P  Q  H  H  K  Q  L  L  L  S  R         p.500

          .         .         .         .         .         .       g.67326
 AAGGCTGCAGCCAAAGAGTCTTTATGCCAGGCAGCCCTGGGTCTCATCCTCAAGGAGAAA       c.1560
 K  A  A  A  K  E  S  L  C  Q  A  A  L  G  L  I  L  K  E  K         p.520

          .         .         .     | 14   .         .         .    g.70526
 GCCATGACCGACACTTTCACTCTTCAGGCACATG | ATCAATTCTCTCCATTTTCGTCTAGC    c.1620
 A  M  T  D  T  F  T  L  Q  A  H  D |   Q  F  S  P  F  S  S  S      p.540

          .         .         .         .         .         .       g.70586
 AGTGGAAGAAGACTGAATATCTCGTATACCAGAAACATGACTCTTAAAGATGGTAAAAAC       c.1680
 S  G  R  R  L  N  I  S  Y  T  R  N  M  T  L  K  D  G  K  N         p.560

   | 15      .         .         .         .         .  | 16      . g.82032
 A | ATGTAGCCATAGCTGTAACGTATAACCATGATGGGTCTTATAGCATGCAG | ATTGAAGAT c.1740
 N |   V  A  I  A  V  T  Y  N  H  D  G  S  Y  S  M  Q   | I  E  D   p.580

          .         .         .         .         .         .       g.82092
 AAAACTTTCCAAGTCCTTGGTAATCTTTACAGCGAGGGAGACTGCACTTACCTGAAATGT       c.1800
 K  T  F  Q  V  L  G  N  L  Y  S  E  G  D  C  T  Y  L  K  C         p.600

          .         .         .         .         .         .       g.82152
 TCTGTTAATGGAGTTGCTAGTAAAGCGAAGCTGATTATCCTGGAAAACACTATTTACCTA       c.1860
 S  V  N  G  V  A  S  K  A  K  L  I  I  L  E  N  T  I  Y  L         p.620

           | 17        .         .         .         .         .    g.84391
 TTTTCCAAG | GAAGGAAGTATTGAGATTGACATTCCAGTCCCCAAATACTTATCTTCTGTG    c.1920
 F  S  K   | E  G  S  I  E  I  D  I  P  V  P  K  Y  L  S  S  V      p.640

          .         .         .         .         .        | 18.    g.87243
 AGCTCACAAGAAACTCAGGGCGGCCCCTTAGCTCCTATGACTGGAACCATTGAAAAG | GTG    c.1980
 S  S  Q  E  T  Q  G  G  P  L  A  P  M  T  G  T  I  E  K   | V      p.660

          .         .         .         .         .         .       g.87303
 TTTGTCAAAGCTGGAGACAAAGTGAAAGCGGGAGATTCCCTCATGGTTATGATCGCCATG       c.2040
 F  V  K  A  G  D  K  V  K  A  G  D  S  L  M  V  M  I  A  M         p.680

           | 19        .         .         .         .         .    g.89062
 AAGATGGAG | CATACCATAAAGTCTCCAAAGGATGGCACAGTAAAGAAAGTGTTCTACAGA    c.2100
 K  M  E   | H  T  I  K  S  P  K  D  G  T  V  K  K  V  F  Y  R      p.700

          .         .         .         .         .         .       g.89122
 GAAGGTGCTCAGGCCAACAGACACACTCCTTTAGTCGAGTTTGAGGAGGAAGAATCAGAC       c.2160
 E  G  A  Q  A  N  R  H  T  P  L  V  E  F  E  E  E  E  S  D         p.720

          .                                                         g.89140
 AAAAGGGAATCGGAATAA                                                 c.2178
 K  R  E  S  E  X                                                   p.725

          .         .         .         .         .         .       g.89200
 actccagcaaggaaatggccagttaagtagtgtcttctctctccaccaaaaagaggaagt       c.*60

          .         .         .         .         .         .       g.89260
 gcctccagcttttctgggggtctcataaagagcagttttactaaatgattgtatgcttat       c.*120

          .         .         .         .         .         .       g.89320
 gctgaacacctttcatattggagaatcatgcatttgggtcactaattatctcaaaatatt       c.*180

          .         .         .         .                           g.89360
 tcatactaataaagttgaattattttttattggaagccaa                           c.*220

 (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 Methylcrotonoyl-CoA carboxylase 1 (alpha) 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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