Complement and inflammasome overactivation mediates paroxysmal nocturnal hemoglobinuria with autoinflammation

Patients with paroxysmal nocturnal hemoglobinuria (PNH) have a clonal population of blood cells deficient in glycosylphosphatidylinositol-anchored (GPI-anchored) proteins, resulting from a mutation in the X-linked gene PIGA. Here we report on a set of patients in whom PNH results instead from bialle...

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Publicado en:J Clin Invest
Autores principales: Höchsmann, Britta, Murakami, Yoshiko, Osato, Makiko, Knaus, Alexej, Kawamoto, Michi, Inoue, Norimitsu, Hirata, Tetsuya, Murata, Shogo, Anliker, Markus, Eggermann, Thomas, Jäger, Marten, Floettmann, Ricarda, Höllein, Alexander, Murase, Sho, Ueda, Yasutaka, Nishimura, Jun-ichi, Kanakura, Yuzuru, Kohara, Nobuo, Schrezenmeier, Hubert, Krawitz, Peter M., Kinoshita, Taroh
Formato: Artigo
Lenguaje:Inglês
Publicado: American Society for Clinical Investigation 2019
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Research Article
Acceso en línea:https://ncbi.nlm.nih.gov/pmc/articles/PMC6877298/
https://ncbi.nlm.nih.gov/pubmed/31430258
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1172/JCI123501
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