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adamts13
ADAMTS13
ADAMTS13 (ADAMTS13; chr9q34.2) is a plasma metalloprotease that cleaves ultra-large von Willebrand factor (ULVWF); ADAMTS13 deficiency (<10% activity) → ULVWF accumulation → platelet microthrombi → TTP. Caplacizumab (anti-VWF nanobody; FDA 2019) is first-line for iTTP.
Entry Metadata
| Field | Value |
|---|---|
| ID | adamts13 |
| Name | ADAMTS13 |
| Status | draft |
| Last reviewed | 2026-06-07 |
| Atlas | 01-human |
| Scale | 03-molecular |
Cross-Atlas Connections
Sources
- Zheng X, Chung D, Takayama TK, et al. Structure of von Willebrand factor-cleaving protease (ADAMTS13), a metalloprotease involved in thrombotic thrombocytopenic purpura. J Biol Chem. 2001;276(44):41059-41063. · PubMed 11557775
- Scully M, Cataland SR, Peyvandi F, et al. Caplacizumab treatment for acquired thrombotic thrombocytopenic purpura. N Engl J Med. 2019;380(4):335-346. · PubMed 30625070
- Peyvandi F, Scully M, Kremer Hovinga JA, et al. Caplacizumab for acquired thrombotic thrombocytopenic purpura. N Engl J Med. 2016;374(6):511-522. · PubMed 26863353