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Publication : A fusion pore phenotype in mast cells of the ruby-eye mouse.

First Author  Oberhauser AF Year  1996
Journal  Proc Natl Acad Sci U S A Volume  93
Issue  25 Pages  14349-54
PubMed ID  8962054 Mgi Jnum  J:37255
Mgi Id  MGI:84657 Doi  10.1073/pnas.93.25.14349
Citation  Oberhauser AF, et al. (1996) A fusion pore phenotype in mast cells of the ruby-eye mouse. Proc Natl Acad Sci U S A 93(25):14349-54
abstractText  Using patch-clamp capacitance and amperometric techniques, we have identified an exocytotic phenotype that affects the function of the fusion pore, the molecular structure that connects the lumen of a secretory vesicle with the extracellular environment during exocytosis. Direct observation of individual exocytotic events in mast cells from the ruby-eye mouse (ru/ru) showed a 3-fold increase in the fraction and duration of transient fusion events with respect to wild-type mice. The fraction of the total fusion events that were transient increased from 0.22 +/- 0.02 (wild type) to 0.65 +/- 0.02 (ru/ru), and the average duration of these events increased from 418 +/- 32 ms (wild type to 1207 +/- 89 ms (ru/ru). We also show that this phenotype can reduce and delay an evoked secretory response by causing the fusion of vesicles that have been previously emptied by repeated cycles of transient fusion, The exocytotic phenotype that we describe here may be a cause of diseases like platelet storage pool deficiency and prolonged bleeding times for which the ruby-eye mouse serves as an animal model, Furthermore, the identification of the gene causing the fusion pore phenotype reported here will illuminate the molecular mechanisms regulating exocytotic fusion.
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