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Anti-cysteine/spacer antibodies that open ADAMTS13 are a common feature in iTTP

Immune-mediated thrombotic thrombocytopenic purpura (iTTP) is caused by an autoantibody-mediated deficiency in ADAMTS13. In healthy individuals, ADAMTS13 has a folded conformation in which the central spacer (S) domain interacts with the C-terminal CUB domains. We recently showed that ADAMTS13 adopt...

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Detalles Bibliográficos
Autores principales: De Waele, Laure, Curie, Alexandre, Kangro, Kadri, Tellier, Edwige, Kaplanski, Gilles, Männik, Andres, Tersteeg, Claudia, Joly, Bérangère S., Coppo, Paul, Veyradier, Agnès, De Meyer, Simon F., Roose, Elien, Vanhoorelbeke, Karen
Formato: Online Artículo Texto
Lenguaje:English
Publicado: American Society of Hematology 2021
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8579268/
https://www.ncbi.nlm.nih.gov/pubmed/34559219
http://dx.doi.org/10.1182/bloodadvances.2021004971