Article
Different NK cell-activating receptors preferentially recruit Rab27a or Munc13-4 to perforin-containing granules for cytotoxicity.
Blood - 5 Nov 2009
Wood Stephanie M, Meeths Marie, Chiang Samuel C C, Bechensteen Anne Grete, Boelens Jaap J, Heilmann Carsten, Horiuchi Hisanori, Rosthøj Steen, Rutynowska Olga, Winiarski Jacek, Stow Jennifer L, Nordenskjöld Magnus, Henter Jan-Inge, Ljunggren Hans-Gustaf, Bryceson Yenan T
Abstract excerpt
The autosomal recessive immunodeficiencies Griscelli syndrome type 2 (GS2) and familial hemophagocytic lymphohistiocytosis type 3 (FHL3) are associated with loss-of-function mutations in RAB27A (encoding Rab27a) and UNC13D (encoding Munc13-4). Munc13-4 deficiency abrogates NK-cell release of perforin-containing lytic granules induced by signals for natural and antibody-dependent cellular cytotoxicity. We...
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