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