Division of Gastroenterology, Children's Hospital, Boston, Massachusetts, USA.
J Clin Invest. 2010 Dec;120(12):4399-4409. doi: 10.1172/JCI42958.
Cholera toxin (CT) causes the massive secretory diarrhea associated with epidemic cholera. To induce disease, CT enters the cytosol of host cells by co-opting a lipid-based sorting pathway from the plasma membrane, through the trans-Golgi network (TGN), and into the endoplasmic reticulum (ER). In the ER, a portion of the toxin is unfolded and retro- translocated to the cytosol. Here, we established zebrafish as a genetic model of intoxication and examined the Derlin and flotillin proteins, which are thought to be usurped by CT for retro-translocation and lipid sorting, respectively. Using antisense morpholino oligomers and siRNA, we found that depletion of Derlin-1, a component of the Hrd-1 retro-translocation complex, was dispensable for CT-induced toxicity. In contrast, the lipid raft-associated proteins flotillin-1 and -2 were required. We found that in mammalian cells, CT intoxication was dependent on the flotillins for trafficking between plasma membrane/endosomes and two pathways into the ER, only one of which appears to intersect the TGN. These results revise current models for CT intoxication and implicate protein scaffolding of lipid rafts in the endo-somal sorting of the toxin-GM1 complex.
霍乱毒素(CT)可引起暴发性分泌性腹泻,与霍乱流行有关。为了引发疾病,CT 通过从质膜采用基于脂质的分选途径,穿过高尔基网络(TGN),进入内质网(ER),进入宿主细胞的细胞质。在 ER 中,部分毒素展开并逆行转运至细胞质。在这里,我们建立了斑马鱼作为中毒的遗传模型,并研究了 Derlin 和 flotillin 蛋白,它们分别被认为是 CT 用于逆行转运和脂质分选的盗用蛋白。通过使用反义的 morpholino 寡核苷酸和 siRNA,我们发现 Hrd-1 逆行转运复合物的组成部分 Derlin-1 的耗竭对于 CT 诱导的毒性是可有可无的。相比之下,脂筏相关蛋白 flotillin-1 和 flotillin-2 是必需的。我们发现,在哺乳动物细胞中,CT 中毒依赖于 flotillin 蛋白在质膜/内体与两条进入 ER 的途径之间的运输,其中只有一条途径似乎与 TGN 相交。这些结果修正了 CT 中毒的现行模型,并表明脂筏的蛋白支架在毒素-GM1 复合物的内体分选中起作用。