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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...
Autores principales: | , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Frontiers Media S.A.
2013
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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 |