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Perforinopathy: A Spectrum of Human Immune Disease Caused by Defective Perforin Delivery or Function

Congenital perforin deficiency is considered a rare cause of human immunopathology and immune dysregulation, and classically presents as a fatal illness early in infancy. However, we propose that a group of related disorders in which killer lymphocytes deliver only partially active perforin or a red...

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Detalles Bibliográficos
Autores principales: Voskoboinik, Ilia, Trapani, Joseph A.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Frontiers Media S.A. 2013
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3860100/
https://www.ncbi.nlm.nih.gov/pubmed/24376445
http://dx.doi.org/10.3389/fimmu.2013.00441