The Physiological Function of von Willebrand's Factor Depends on Its Tubular Storage in Endothelial Weibel-Palade Bodies
作者： Grégoire Michaux , Kate B. Abbitt , Lucy M. Collinson , Sandra L. Haberichter , Keith E. Norman
DOI: 10.1016/J.DEVCEL.2005.12.012
关键词:
摘要: Weibel-Palade bodies are the 1-5 microm long rod-shaped storage organelles of endothelial cells. We have investigated determinants and functional significance this shape. find that folding hemostatic protein von Willebrand's factor (VWF) into tubules underpins rod-like shape bodies. Further, while propeptide N-terminal domains mature VWF sufficient to form tubules, their maintenance relies on a pH-dependent interaction between two. show tubular conformation is essential for rapid unfurling 100 long, platelet-catching filaments when exposed neutral pH after exocytosis in cell culture living blood vessels. If disassembled prior exocytosis, then short or tangled released platelet recruitment reduced. Thus, 100-fold compaction determines unique critical protein's function.
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