Getting secretory granules ready for prime time.
Several rare human diseases have shed important light on the secretory pathway required for lymphocyte cytotoxicity. In this issue of Cell, Feldmann et al. identify mutations in Munc13-4 as a cause of familial hemophagocytic lymphohistiocytosis. Munc13-4 appears to be involved in the priming of cytotoxic granules prior to fusion with the plasma membrane.
Zhang
Bin
B
Howard Hughes Medical Institute and Department of Internal Medicine, University of Michigan, Ann Arbor, MI 48109, USA.
Ginsburg
David
D
D000818:Animals; D015550:Cell Degranulation; D002462:Cell Membrane / Q000378:metabolism; D005089:Exocytosis; D015616:Histiocytosis, Non-Langerhans-Cell / Q000235:genetics* / Q000378:metabolism / Q000473:pathology; D006801:Humans; D008561:Membrane Fusion; D009154:Mutation; D011506:Proteins / Q000235:genetics / Q000378:metabolism*; D022142:Secretory Vesicles / Q000378:metabolism*; D013602:T-Lymphocytes, Cytotoxic / Q000166:cytology / Q000276:immunology
D011506:Proteins; C430713:Unc13d protein, rat