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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Bibliographic Details
Main Authors: Joseph A. Trapani, Ilia eVoskoboinik
Format: Article
Language:English
Published: Frontiers Media S.A. 2013-12-01
Series:Frontiers in Immunology
Subjects:
Online Access:http://journal.frontiersin.org/Journal/10.3389/fimmu.2013.00441/full