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Temperature sensitivity of human perforin mutants unmasks subtotal loss of cytotoxicity, delayed FHL, and a predisposition to cancer

The pore-forming protein perforin is critical for defense against many human pathogens and for preventing a catastrophic collapse of immune homeostasis, manifested in infancy as Type 2 familial hemophagocytic lymphohistiocytosis (FHL). However, no evidence has yet linked defective perforin cytotoxic...

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Detalhes bibliográficos
Main Authors: Chia, Jenny, Yeo, Kim Pin, Whisstock, James C., Dunstone, Michelle A., Trapani, Joseph A., Voskoboinik, Ilia
Formato: Artigo
Idioma:Inglês
Publicado em: National Academy of Sciences 2009
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Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC2701033/
https://ncbi.nlm.nih.gov/pubmed/19487666
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1073/pnas.0903815106
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