hydroxyacyl-CoA dehydrogenase/3-ketoacyl-CoA thiolase/enoyl-CoA hydratase (trifunctional protein), alpha subunit (HADHA) - coding DNA reference sequence

(used for variant description)

(last modified October 12, 2018)


This file was created to facilitate the description of sequence variants on transcript NM_000182.4 in the HADHA gene based on a coding DNA reference sequence following the HGVS recommendations.

The sequence was taken from NG_007121.1, covering HADHA transcript NM_000182.4.


Please note that introns are available by clicking on the exon numbers above the sequence.
 (upstream sequence)
                                                   .                g.4905
                                                   cggccactcg       c.-121

 .         .         .         .         .         .                g.4965
 gcatctgggttttagtcaggcagtgctccaggcgcctggctgccaggctacgggagaaaa       c.-61

 .         .         .         .         .         .                g.5025
 gtcctccgctcggccgcacccgttagaggcgctctccactgctgtcctcttcagctcaag       c.-1

          .         .         .         .         .         .       g.5085
 ATGGTGGCCTGCCGGGCGATTGGCATCCTCAGCCGCTTTTCTGCCTTCAGGATCCTCCGC       c.60
 M  V  A  C  R  A  I  G  I  L  S  R  F  S  A  F  R  I  L  R         p.20

         | 02.         .         .         .          | 03        . g.10628
 TCCCGAG | GTTATATATGCCGCAATTTTACAGGGTCTTCTGCTTTGCTGA | CCAGAACCCAT c.120
 S  R  G |   Y  I  C  R  N  F  T  G  S  S  A  L  L  T |   R  T  H   p.40

          .         .         .         .         .         .       g.10688
 ATTAACTATGGAGTCAAAGGGGATGTGGCAGTTGTTCGAATTAACTCTCCCAATTCAAAG       c.180
 I  N  Y  G  V  K  G  D  V  A  V  V  R  I  N  S  P  N  S  K         p.60

  | 04       .         .         .         .         .         .    g.12693
  | GTAAATACACTGAGTAAAGAGCTACATTCAGAGTTCTCAGAAGTTATGAATGAAATCTGG    c.240
  | V  N  T  L  S  K  E  L  H  S  E  F  S  E  V  M  N  E  I  W      p.80

          .         .         .         .         .         .       g.12753
 GCTAGTGATCAAATCAGAAGTGCCGTCCTTATCTCATCAAAGCCAGGCTGCTTTATTGCA       c.300
 A  S  D  Q  I  R  S  A  V  L  I  S  S  K  P  G  C  F  I  A         p.100

          .     | 05   .         .         .         .         .    g.15312
 GGTGCTGATATCAA | CATGTTAGCCGCTTGCAAGACCCTTCAAGAAGTAACACAGCTATCA    c.360
 G  A  D  I  N  |  M  L  A  A  C  K  T  L  Q  E  V  T  Q  L  S      p.120

          .         .         .         .         .         .       g.15372
 CAAGAAGCACAGAGAATAGTTGAGAAACTTGAAAAGTCCACAAAGCCTATTGTGGCTGCC       c.420
 Q  E  A  Q  R  I  V  E  K  L  E  K  S  T  K  P  I  V  A  A         p.140

          .         .         .    | 06    .         .         .    g.17369
 ATCAATGGATCCTGCCTGGGAGGAGGACTTGAG | GTTGCCATTTCATGCCAATACAGAATA    c.480
 I  N  G  S  C  L  G  G  G  L  E   | V  A  I  S  C  Q  Y  R  I      p.160

          .         .         .         .         .         .       g.17429
 GCAACAAAAGACAGAAAAACAGTATTAGGTACCCCTGAAGTTTTGCTGGGGGCCTTACCA       c.540
 A  T  K  D  R  K  T  V  L  G  T  P  E  V  L  L  G  A  L  P         p.180

          .         .         .    | 07    .         .         .    g.19354
 GGAGCAGGAGGCACACAAAGGCTGCCCAAAATG | GTGGGTGTGCCTGCTGCTTTGGACATG    c.600
 G  A  G  G  T  Q  R  L  P  K  M   | V  G  V  P  A  A  L  D  M      p.200

          .         .         .         .         .         .       g.19414
 ATGCTGACTGGTAGAAGCATTCGTGCAGACAGGGCAAAGAAAATGGGACTGGTTGACCAA       c.660
 M  L  T  G  R  S  I  R  A  D  R  A  K  K  M  G  L  V  D  Q         p.220

          .       | 08 .         .         .         .         .    g.34489
 CTGGTGGAACCCCTGG | GACCAGGACTAAAACCTCCAGAGGAACGGACAATAGAATACCTA    c.720
 L  V  E  P  L  G |   P  G  L  K  P  P  E  E  R  T  I  E  Y  L      p.240

          .         .         .         .         .         .       g.34549
 GAAGAAGTTGCAATTACTTTTGCCAAAGGACTAGCTGATAAGAAGATCTCTCCAAAGAGA       c.780
 E  E  V  A  I  T  F  A  K  G  L  A  D  K  K  I  S  P  K  R         p.260

          .          | 09        .         .         .         .    g.35100
 GACAAGGGATTGGTGGAAA | AATTGACAGCGTATGCCATGACTATTCCATTTGTCAGGCAA    c.840
 D  K  G  L  V  E  K |   L  T  A  Y  A  M  T  I  P  F  V  R  Q      p.280

          .         .         .         .         .         .       g.35160
 CAGGTTTACAAAAAAGTGGAAGAAAAAGTGCGAAAGCAGACTAAAGGCCTTTATCCTGCA       c.900
 Q  V  Y  K  K  V  E  E  K  V  R  K  Q  T  K  G  L  Y  P  A         p.300

          .         | 10         .         .         .         .    g.37036
 CCTCTGAAAATAATTGAT | GTGGTAAAGACTGGAATTGAGCAAGGGAGTGATGCCGGTTAT    c.960
 P  L  K  I  I  D   | V  V  K  T  G  I  E  Q  G  S  D  A  G  Y      p.320

          .      | 11  .         .         .         .         .    g.39776
 CTCTGTGAATCTCAG | AAATTTGGAGAGCTTGTAATGACCAAAGAATCAAAGGCCTTGATG    c.1020
 L  C  E  S  Q   | K  F  G  E  L  V  M  T  K  E  S  K  A  L  M      p.340

          .         .         .         .         .         .       g.39836
 GGACTCTACCATGGTCAGGTCCTGTGCAAGAAGAATAAATTTGGAGCTCCACAGAAGGAT       c.1080
 G  L  Y  H  G  Q  V  L  C  K  K  N  K  F  G  A  P  Q  K  D         p.360

       | 12  .         .         .         .         .         .    g.45479
 GTTAA | GCATCTGGCTATTCTTGGTGCAGGGCTGATGGGAGCAGGCATCGCCCAAGTCTCC    c.1140
 V  K  |  H  L  A  I  L  G  A  G  L  M  G  A  G  I  A  Q  V  S      p.380

          .         .         .         .         .         .       g.45539
 GTGGATAAGGGGCTAAAGACTATACTTAAAGATGCCACCCTCACTGCGCTAGACCGAGGA       c.1200
 V  D  K  G  L  K  T  I  L  K  D  A  T  L  T  A  L  D  R  G         p.400

          .         . | 13       .         .         .         .    g.48340
 CAGCAACAAGTGTTCAAAGG | ATTGAATGACAAAGTGAAGAAGAAAGCTCTAACATCATTT    c.1260
 Q  Q  Q  V  F  K  G  |  L  N  D  K  V  K  K  K  A  L  T  S  F      p.420

          .         .         .         .         .         .       g.48400
 GAAAGGGATTCCATCTTCAGCAACTTGACTGGGCAGCTTGATTACCAAGGTTTTGAAAAG       c.1320
 E  R  D  S  I  F  S  N  L  T  G  Q  L  D  Y  Q  G  F  E  K         p.440

          .         .         .         .         .         .       g.48460
 GCCGACATGGTGATTGAAGCTGTGTTTGAGGACCTTAGTCTTAAGCACAGAGTGCTAAAG       c.1380
 A  D  M  V  I  E  A  V  F  E  D  L  S  L  K  H  R  V  L  K         p.460

          .   | 14     .         .         .         .         .    g.51891
 GAAGTAGAAGCG | GTGATTCCAGATCACTGTATCTTTGCCAGTAACACATCTGCTCTCCCA    c.1440
 E  V  E  A   | V  I  P  D  H  C  I  F  A  S  N  T  S  A  L  P      p.480

          .         .         .          | 15        .         .    g.54409
 ATCAGTGAAATCGCTGCTGTCAGCAAAAGACCTGAGAAG | GTGATTGGCATGCACTACTTC    c.1500
 I  S  E  I  A  A  V  S  K  R  P  E  K   | V  I  G  M  H  Y  F      p.500

          .         .         .         .         .         .       g.54469
 TCTCCCGTGGACAAGATGCAGCTGCTGGAGATTATCACGACCGAGAAAACTTCCAAAGAC       c.1560
 S  P  V  D  K  M  Q  L  L  E  I  I  T  T  E  K  T  S  K  D         p.520

          .         .         .         .         .         .       g.54529
 ACCAGTGCTTCAGCTGTAGCAGTTGGTCTCAAGCAGGGGAAGGTCATCATTGTGGTTAAG       c.1620
 T  S  A  S  A  V  A  V  G  L  K  Q  G  K  V  I  I  V  V  K         p.540

  | 16       .         .         .         .         .         .    g.55042
  | GATGGACCTGGCTTCTATACTACCAGGTGTCTTGCGCCCATGATGTCTGAAGTCATCCGA    c.1680
  | D  G  P  G  F  Y  T  T  R  C  L  A  P  M  M  S  E  V  I  R      p.560

           | 17        .         .         .         .         .    g.55899
 ATCCTCCAG | GAAGGAGTTGACCCGAAGAAGCTGGATTCCCTGACCACAAGCTTTGGCTTT    c.1740
 I  L  Q   | E  G  V  D  P  K  K  L  D  S  L  T  T  S  F  G  F      p.580

          .         .         .         .         .         .       g.55959
 CCTGTGGGTGCCGCCACACTGGTGGATGAAGTTGGTGTGGATGTAGCGAAACATGTGGCG       c.1800
 P  V  G  A  A  T  L  V  D  E  V  G  V  D  V  A  K  H  V  A         p.600

          .         .         .         .         .         .       g.56019
 GAAGATCTGGGCAAAGTCTTTGGGGAGCGGTTTGGAGGTGGAAACCCAGAACTGCTGACA       c.1860
 E  D  L  G  K  V  F  G  E  R  F  G  G  G  N  P  E  L  L  T         p.620

          .         .      | 18  .         .         .         .    g.57231
 CAGATGGTGTCCAAGGGCTTCCTAG | GTCGTAAATCTGGGAAGGGCTTTTACATCTATCAG    c.1920
 Q  M  V  S  K  G  F  L  G |   R  K  S  G  K  G  F  Y  I  Y  Q      p.640

          .         .         .         .         .         .       g.57291
 GAGGGTGTGAAGAGGAAGGATTTGAATTCTGACATGGATAGTATTTTAGCGAGTCTGAAG       c.1980
 E  G  V  K  R  K  D  L  N  S  D  M  D  S  I  L  A  S  L  K         p.660

          .         . | 19       .         .         .         .    g.58032
 CTGCCTCCTAAGTCTGAAGT | CTCATCAGACGAAGACATCCAGTTCCGCCTGGTGACAAGA    c.2040
 L  P  P  K  S  E  V  |  S  S  D  E  D  I  Q  F  R  L  V  T  R      p.680

          .         .         .         .         .         .       g.58092
 TTTGTGAATGAGGCAGTCATGTGCCTGCAAGAGGGGATCTTGGCCACACCTGCAGAGGGA       c.2100
 F  V  N  E  A  V  M  C  L  Q  E  G  I  L  A  T  P  A  E  G         p.700

          .         .         .         .       | 20 .         .    g.58239
 GACATCGGAGCCGTCTTTGGGCTTGGCTTCCCGCCTTGTCTGGGAG | GGCCTTTCCGCTTT    c.2160
 D  I  G  A  V  F  G  L  G  F  P  P  C  L  G  G |   P  F  R  F      p.720

          .         .         .         .         .         .       g.58299
 GTGGATCTGTATGGCGCCCAGAAGATAGTGGACCGGCTCAAGAAATATGAAGCTGCCTAT       c.2220
 V  D  L  Y  G  A  Q  K  I  V  D  R  L  K  K  Y  E  A  A  Y         p.740

          .         .         .         .         .         .       g.58359
 GGAAAACAGTTCACCCCATGCCAGCTGCTAGCTGACCATGCTAACAGCCCTAACAAGAAG       c.2280
 G  K  Q  F  T  P  C  Q  L  L  A  D  H  A  N  S  P  N  K  K         p.760

          .                                                         g.58371
 TTCTACCAGTGA                                                       c.2292
 F  Y  Q  X                                                         p.763

          .         .         .         .         .         .       g.58431
 gcaggcctcatgcctcgctcagtcagtgcactaaccccagctgccggcagtgctggttct       c.*60

          .         .         .         .         .         .       g.58491
 ccaacagagtggtgtctagatttatcagagtaacgagaagacaaactccggcactgggtt       c.*120

          .         .         .         .         .         .       g.58551
 tgctccctgattaaagtgccttcagccaagaccatctctccctcctggtgaagtgtgact       c.*180

          .         .         .         .         .         .       g.58611
 tcgaattagtttgcacttcctgttggaaggtagagcccactgctcattgtataagccccg       c.*240

          .         .         .         .         .         .       g.58671
 aggcctagagtggcagccaagagccatctgaagccacctctctgcctgttcctcccaaga       c.*300

          .         .         .         .         .         .       g.58731
 ggccagggtggccaggggtggtgagggcagttctgcacccagccaaacacataacaataa       c.*360

          .         .         .         .         .         .       g.58791
 aaaccaaactctgtgtcagcatctttgcccttctggtttaaacgcctccttcaaaaagca       c.*420

          .         .         .         .         .         .       g.58851
 atctggaagaaagccctgtgctttgggggagtaagaatgtgtgtgcagaattctaggcag       c.*480

          .         .         .         .         .         .       g.58911
 caccttagggagggactgggatgagagaaagtgggacctggtgggctcaaccacacacac       c.*540

          .         .         .         .         .         .       g.58971
 ctgtctgtgcagatgctttgcccaggcttctcaccacggtgtaccgggatattaaacctc       c.*600

          .                                                         g.58986
 tttccccagcctgga                                                    c.*615

 (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 Hydroxyacyl-CoA dehydrogenase/3-ketoacyl-CoA thiolase/enoyl-CoA hydratase (trifunctional protein), alpha subunit 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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