18.STX11介导淋巴细胞分泌及细胞毒作用.pdfVIP

T H F E JOURNALO IMMUNOLOGY CUTTING EDGE Cutting Edge: Syntaxin 11 Regulates Lymphocyte-Mediated Secretion and Cytotoxicity1 Laura N. Arneson,2 Adipong Brickshawana, Colin M. Segovis, Renee A. Schoon, Christopher J. Dick, and Paul J. Leibson Little is known about the regulatory roles of specific solu- Mutations in the exocytic machinery (e.g., Munc13-4 and ble N-ethylmaleimide-sensitive factor attachment protein Rab27a) have also been shown to result in FHL by impairing receptor (SNARE) proteins in cytotoxic lymphocytes. Re- the ability to deliver granules correctly (4, 5). cent information suggests that mutations in the SNARE Most recently, mutations in the soluble N-ethylmaleimide- protein syntaxin 11 result in a form of familial hemoph- sensitive factor attachment protein receptor (SNARE) protein agocytic lymphohistiocytosis (FHL). Because genetic ab- syntaxin 11 have been shown to be responsible for a specific normalities in key granule components (e.g., perforin) subtype of FHL (6, 7). Surprisingly, in contrast to other known or in regulators of secretion (e.g., Munc13– 4) underlie causes of FHL, syntaxin 11 is not known to be involved in the secretory machinery or the cytotoxic arsenal of NK cells or the other identified forms of FHL, we assessed whether CTLs (6, 8). Moreover, it is unclear from initial character