Defective cytotoxic lymphocyte degranulation in syntaxin-11–deficient familial hemophagocytic lymphohistiocytosis 4 (FHL4) patients

Familial hemophagocytic lymphohistiocytosis (FHL) is typically an early onset, fatal disease characterized by a sepsislike illness with cytopenia, hepatosplenomegaly, and deficient lymphocyte cytotoxicity. Disease-causing mutations have been identified in genes encoding perforin (PRF1/FHL2), Munc13-...

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Main Authors: Bryceson, Yenan T., Rudd, Eva, Zheng, Chengyun, Edner, Josefine, Ma, Daoxin, Wood, Stephanie M., Bechensteen, Anne Grete, Boelens, Jaap J., Celkan, Tiraje, Farah, Roula A., Hultenby, Kjell, Winiarski, Jacek, Roche, Paul A., Nordenskjöld, Magnus, Henter, Jan-Inge, Long, Eric O., Ljunggren, Hans-Gustaf
Formato: Artigo
Idioma:Inglês
Publicado: American Society of Hematology 2007
Assuntos:
Immunobiology
Acceso en liña:https://ncbi.nlm.nih.gov/pmc/articles/PMC1976360/
https://ncbi.nlm.nih.gov/pubmed/17525286
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1182/blood-2007-02-074468
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