Article
Altered distribution and function of natural killer cells in murine and human Niemann-Pick disease type C1.
Blood - 2 Jan 2014
Speak Anneliese O, Te Vruchte Danielle, Davis Lianne C, Morgan Anthony J, Smith David A, Yanjanin Nicole M, Simmons Louise, Hartung Ralf, Runz Heiko, Mengel Eugen, Beck Michael, Imrie Jackie, Jacklin Elizabeth, Wraith James E, Hendriksz Christian, Lachmann Robin, Cognet Celine, Sidhu Rohini, Fujiwara Hideji, Ory Daniel S, Galione Antony, Porter Forbes D, Vivier Eric, Platt Frances M
Abstract excerpt
Niemann-Pick type C (NPC) is a neurodegenerative lysosomal storage disorder caused by defects in the lysosomal proteins NPC1 or NPC2. NPC cells are characterized by reduced lysosomal calcium levels and impaired sphingosine transport from lysosomes. Natural killer (NK) cells kill virally infected/transformed cells via degranulation of lysosome-related organelles. Their trafficking from lymphoid tissues into the...
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