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Gene

ADAMTS13

ADAM metallopeptidase with thrombospondin type 1 motif 13

Summary

Cytogenetic location
9q34.2
Coordinates
Chromosome 9: 133,422,367–133,459,386 (GRCh38)
Exons
29 exons across 37 kb
Protein
1,371 amino acids
Inheritance
Autosomal recessive
Also known as
VWFCP, TTP, vWF-CP, FLJ42993, MGC118899, MGC118900, DKFZp434C2322
Previous symbols
C9orf8
MANE Select transcript
ENST00000355699.7NM_139027.6
Locus type
gene with protein product
  • Very high
  • Confident
  • Low
  • Very low

Reference genomeGRCh38

Positions on gene and variant pages follow this choice, in this browser.

Curated reports relevant to this gene

  • Congenital Thrombotic Thrombocytopenic Purpura

    • Congenital Thrombotic Thrombocytopenic Purpura (Upshaw-Schulman Syndrome)Autosomal recessive

      Caused by changes in both copies of ADAMTS13; episodes may begin in newborns, often with severe jaundice, or first appear in adulthood, frequently triggered by pregnancy or infection.

      • ClinGen rates the link definitive
      • 4 GenCC submitters rate it definitive or strong
      What knowing changes

      Regular preventive treatment with plasma or recombinant ADAMTS13 prevents acute episodes, and close monitoring is needed in pregnancy.

  • Inherited Thrombocytopenias

    • Inherited Thrombocytopenia

      A group of conditions causing a lifelong low platelet count, linked to genes such as MYH9, ANKRD26, ETV6, WAS, MPL, RBM8A, GATA1 and MECOM, and inherited in dominant, recessive or X-linked patterns.

      • ClinGen rates the link definitive
      • 4 GenCC submitters rate it definitive or strong
      What knowing changes

      A genetic diagnosis avoids misdiagnosis as immune thrombocytopenia, with unnecessary steroids or spleen removal, and guides monitoring for related kidney, hearing or blood problems.

Inheritance

Autosomal recessive
It takes a changed copy from each parent. With one, you are a carrier and usually unaffected; when both parents are carriers, each child has a one-in-four chance of inheriting two.

How ADAMTS13 is laid out

  • Coding sequence
  • Untranslated region
  • Intron
  • Direction of transcription
  • Known disease-causing sites
  • Stretches short reads find hard
Chromosome 9, with a bracket where ADAMTS13 sits. ADAMTS13 and 25 genes near it on chromosome 9, from 132,940,877 to 133,940,876.Chromosome 99q34.2p24.3p24.2p24.1p23p22.3p22.2p22.1p21.3p21.2p21.1p13.3p13.2p13.1p12p11.2p11.1q11q12q13q21.11q21.12q21.13q21.2q21.31q21.32q21.33q22.1q22.2q22.31q22.32q22.33q31.1q31.2q31.3q32q33.1q33.2q33.3q34.11q34.12q34.13q34.2q34.3133 Mb133.2 Mb133.4 Mb133.6 Mb133.8 MbTSC1: 132,891,348–132,946,874, reverse strandTSC1GFI1B: 132,944,000–132,991,687, forward strandGFI1BGTF3C5: 133,030,675–133,058,503, forward strandGTF3C5CEL: 133,061,981–133,071,861, forward strandCELRALGDS: 133,097,657–133,149,440, reverse strandRALGDS: 133,098,121–133,163,914, reverse strandGBGT1: 133,151,020–133,164,245, reverse strandGBGT1OBP2B: 133,205,277–133,209,309, reverse strandOBP2BABO: 133,233,278–133,276,024, reverse strandABOSURF6: 133,328,684–133,336,289, reverse strandMED22: 133,338,312–133,348,140, reverse strandRPL7A: 133,347,950–133,351,435, forward strandSURF1: 133,351,069–133,356,676, reverse strandSURF1SURF2: 133,356,520–133,362,917, forward strandSURF2SURF4: 133,361,450–133,376,233, reverse strandSURF4STKLD1: 133,376,332–133,406,096, forward strandSTKLD1REXO4: 133,406,026–133,418,096, reverse strandREXO4ADAMTS13: 133,422,367–133,459,386, forward strandADAMTS13CACFD1: 133,459,965–133,470,848, forward strandCACFD1SLC2A6: 133,462,053–133,479,148, reverse strandSLC2A6MYMK: 133,514,586–133,528,612, reverse strandMYMKADAMTSL2: 133,532,164–133,575,522, forward strandADAMTSL2FAM163B: 133,577,076–133,609,420, reverse strandFAM163BDBH: 133,634,456–133,659,329, forward strandDBHSARDH: 133,663,528–133,739,976, reverse strandSARDHVAV2: 133,761,892–133,992,854, reverse strandVAV2Diagram of ADAMTS13: 29 exons on the forward strand of chromosome 9Exon 1: 2 known disease-causing sitesExon 2: 1 known disease-causing siteExon 3: 3 known disease-causing sitesExon 4: 1 known disease-causing siteExon 6: 2 known disease-causing sitesExon 7: 4 known disease-causing sitesExon 8: 1 known disease-causing siteExon 9: 2 known disease-causing sitesExon 10: 4 known disease-causing sitesExon 11: 2 known disease-causing sitesExon 13: 2 known disease-causing sitesExon 16: 2 known disease-causing sitesExon 17: 3 known disease-causing sitesExon 18: 2 known disease-causing sitesExon 19: 2 known disease-causing sitesExon 20: 1 known disease-causing siteExon 23: 3 known disease-causing sitesExon 24: 4 known disease-causing sitesExon 25: 2 known disease-causing sitesExon 26: 4 known disease-causing sitesExon 27: 2 known disease-causing sitesExon 29: 1 known disease-causing siteExon 1: 133,422,367–133,422,548, 182 basesExon 2: 133,423,101–133,423,167, 67 basesExon 3: 133,424,321–133,424,478, 158 basesExon 4: 133,425,529–133,425,612, 84 basesExon 5: 133,425,938–133,426,062, 125 basesExon 6: 133,426,199–133,426,345, 147 basesExon 7: 133,428,634–133,428,771, 138 basesExon 8: 133,429,939–133,430,101, 163 basesExon 9: 133,432,588–133,432,692, 105 basesExon 10: 133,433,378–133,433,529, 152 basesExon 11: 133,433,641–133,433,704, 64 basesExon 12: 133,436,829–133,436,955, 127 basesExon 13: 133,437,749–133,437,897, 149 basesExon 14: 133,438,246–133,438,366, 121 basesExon 15: 133,439,366–133,439,446, 81 basesExon 16: 133,440,344–133,440,525, 182 basesExon 17: 133,442,399–133,442,534, 136 basesExon 18: 133,442,614–133,442,743, 130 basesExon 19: 133,443,376–133,443,561, 186 basesExon 20: 133,444,863–133,445,052, 190 basesExon 21: 133,445,699–133,445,819, 121 basesExon 22: 133,448,599–133,448,728, 130 basesExon 23: 133,449,783–133,449,965, 183 basesExon 24: 133,454,415–133,454,619, 205 basesExon 25: 133,455,285–133,455,435, 151 basesExon 26: 133,456,069–133,456,215, 147 basesExon 27: 133,456,543–133,456,719, 177 basesExon 28: 133,457,910–133,458,094, 185 basesExon 29: 133,458,974–133,459,386, 413 bases1246810121416182022242628295′3′

Exons of the gene's MANE Select transcript (release v1.5), from NCBI and EMBL-EBI, on the GRCh38 reference genome.

Chromosome bands from UCSC's GRCh38 ideogram (2022-10-29).

Nearby genes from Ensembl release 116, on GRCh38.

Exons make up 4.4 kb of the gene's 37 kb; 4,116 of those bases code for the protein.

How a genome test reads ADAMTS13

59

positions in ADAMTS13 where ClinVar classes a change as pathogenic or likely pathogenic

None of them falls in a region short reads find hard.

A whole genome covers all 37 kb of ADAMTS13, introns included, not only the 4.4 kb of exons.

Where this comes from

  • HGNC: the gene's symbol, name, other symbols and location.
  • MANE Select v1.5, from NCBI and EMBL-EBI: the transcript, its exons and coding sequence.
  • GenCC and ClinGen, released 2026-10-16: which conditions the gene is linked to, and how strongly.
  • The ACMG secondary findings list, SF v3.2.
  • ClinVar and the GIAB stratifications: known disease-causing sites, and the regions short reads find hard.
  • The Human Phenotype Ontology, release 2026-09-01: the traits linked to the gene. Find out more at hpo.jax.org.
  • Data release 2026.10.16-c6eea6bf.

Cite this page

APA

Dante Labs. (n.d.). ADAMTS13 gene: ADAM metallopeptidase with thrombospondin type 1 motif 13. Retrieved 17 October 2026, from https://dantelabs.com/genes/adamts13

Vancouver

Dante Labs. ADAMTS13 gene: ADAM metallopeptidase with thrombospondin type 1 motif 13 [Internet]. [cited 17 October 2026]. Available from: https://dantelabs.com/genes/adamts13

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