Constitutive activation of WASp in X-linked neutropenia renders neutrophils hyperactive

Congenital neutropenia is characterized by low absolute neutrophil numbers in blood, leading to recurrent bacterial infections, and patients often require life-long granulocyte CSF (G-CSF) support. X-linked neutropenia (XLN) is caused by gain-of-function mutations in the actin regulator Wiskott-Aldr...

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Dades bibliogràfiques
Publicat a:J Clin Invest
Autors principals: Keszei, Marton, Record, Julien, Kritikou, Joanna S., Wurzer, Hannah, Geyer, Chiara, Thiemann, Meike, Drescher, Paul, Brauner, Hanna, Köcher, Laura, James, Jaime, He, Minghui, Baptista, Marisa A.P., Dahlberg, Carin I.M., Biswas, Amlan, Lain, Sonia, Lane, David P., Song, Wenxia, Pütsep, Katrin, Vandenberghe, Peter, Snapper, Scott B., Westerberg, Lisa S.
Format: Artigo
Idioma:Inglês
Publicat: American Society for Clinical Investigation 2018
Matèries:
Research Article
Accés en línia:https://ncbi.nlm.nih.gov/pmc/articles/PMC6118594/
https://ncbi.nlm.nih.gov/pubmed/30124469
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1172/JCI64772
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