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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Bibliografiske detaljer
Udgivet i:J Clin Invest
Main Authors: 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
Sprog:Inglês
Udgivet: American Society for Clinical Investigation 2018
Fag:
Research Article
Online adgang: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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