Article
Adaptor protein 3-dependent microtubule-mediated movement of lytic granules to the immunological synapse.
Nature immunology - 1 Nov 2003
Clark Richard H, Stinchcombe Jane C, Day Anna, Blott Emma, Booth Sarah, Bossi Giovanna, Hamblin Terry, Davies E Graham, Griffiths Gillian M
Abstract excerpt
Hermansky-Pudlak syndrome (HPS) is a rare autosomal recessive disease characterized by platelet defects and oculocutaneous albinism. Individuals with HPS type 2 (HPS2) lack the cytosolic adaptor protein 3 (AP-3) involved in lysosomal sorting, and are also immunodeficient. Here we characterize an HPS2 mutation and demonstrate that AP-3 deficiency leads to a loss of cytotoxic T lymphocyte (CTL)-mediated...
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