Spessott Waldo A, Sanmillan Maria L, McCormick Margaret E, Kulkarni Vineet V, Giraudo Claudio G
Division of Cell Pathology, Department of Pathology and Laboratory Medicine, University of Pennsylvania-Children's Hospital of Philadelphia, Philadelphia PA 19104.
Biomedical Graduate Studies, University of Pennsylvania, Philadelphia, PA 19104.
Proc Natl Acad Sci U S A. 2017 Mar 14;114(11):E2176-E2185. doi: 10.1073/pnas.1617981114. Epub 2017 Mar 6.
The atypical lipid-anchored Syntaxin 11 (STX11) and its binding partner, the Sec/Munc (SM) protein Munc18-2, facilitate cytolytic granule release by cytotoxic T lymphocytes (CTLs) and natural killer (NK) cells. Patients carrying mutations in these genes develop familial hemophagocytic lymphohistiocytosis, a primary immunodeficiency characterized by impaired lytic granule exocytosis. However, whether a SNARE such as STX11, which lacks a transmembrane domain, can support membrane fusion in vivo is uncertain, as is the precise role of Munc18-2 during lytic granule exocytosis. Here, using a reconstituted "flipped" cell-cell fusion assay, we show that lipid-anchored STX11 and its cognate SNARE proteins mainly support exchange of lipids but not cytoplasmic content between cells, resembling hemifusion. Strikingly, complete fusion is stimulated by addition of wild-type Munc18-2 to the assay, but not of Munc18-2 mutants with abnormal STX11 binding. Our data reveal that Munc18-2 is not just a chaperone of STX11 but also directly contributes to complete membrane merging by promoting SNARE complex assembly. These results further support the concept that SM proteins in general are part of the core fusion machinery. This fusion mechanism likely contributes to other cell-type-specific exocytic processes such as platelet secretion.
非典型脂质锚定的Syntaxin 11(STX11)及其结合伴侣Sec/Munc(SM)蛋白Munc18-2,促进细胞毒性T淋巴细胞(CTL)和自然杀伤(NK)细胞释放溶细胞颗粒。携带这些基因突变的患者会发展为家族性噬血细胞性淋巴组织细胞增生症,这是一种以溶细胞颗粒胞吐功能受损为特征的原发性免疫缺陷病。然而,像STX11这样缺乏跨膜结构域的SNARE蛋白是否能在体内支持膜融合尚不确定,Munc18-2在溶细胞颗粒胞吐过程中的精确作用也不明确。在这里,我们使用重组的“翻转”细胞-细胞融合试验表明,脂质锚定的STX11及其同源SNARE蛋白主要支持细胞间脂质交换,而非细胞质内容物交换,类似于半融合。令人惊讶的是,向试验中添加野生型Munc18-2可刺激完全融合,但添加与STX11结合异常的Munc18-2突变体则不能。我们的数据表明,Munc18-2不仅是STX11的伴侣蛋白,还通过促进SNARE复合体组装直接促成完全的膜融合。这些结果进一步支持了SM蛋白通常是核心融合机制一部分的概念。这种融合机制可能有助于其他细胞类型特异性的胞吐过程,如血小板分泌。