Defective Granule Exocytosis in Rab27a-Deficient Lymphocytes from Ashen Mice
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چکیده
منابع مشابه
Defective Granule Exocytosis in Rab27a-Deficient Lymphocytes from Ashen Mice
Because mutations in Rab27a have been linked to immune defects in humans, we have examined cytotoxic lymphocyte function in ashen mice, which contain a splicing mutation in Rab27a. Ashen cytotoxic T lymphocytes (CTLs) showed a >90% reduction in lytic activity on Fas-negative target cells compared with control C3H CTLs, and ashen natural killer cell activity was likewise diminished. Although the...
متن کاملKinetics of Rab27a-dependent actions on vesicle docking and priming in pancreatic beta-cells.
The small GTPase Rab27a, along with the isoforms of Rab3, is present on insulin secretory granules and has been implicated in regulation of Ca(2+)-triggered exocytosis. We have used membrane capacitance measurements to define the role of Rab27a in regulating the size and refilling of distinct pools of insulin granules by comparison of evoked secretory responses from Rab27a-null ashen and strain...
متن کاملRab27a mediates the tight docking of insulin granules onto the plasma membrane during glucose stimulation.
The monomeric small GTPase Rab27a is specifically localized on both secretory granules and lysosome-related organelles. Although natural mutations of the Rab27a gene in human Griscelli syndrome and in ashen mice cause partial albinism and immunodeficiency reflecting the dysfunction of lysosome-related organelles, phenotypes resulting from the defective exocytosis of secretory granules have not ...
متن کاملHEMOSTASIS, THROMBOSIS, AND VASCULAR BIOLOGY The regulation of platelet-dense granules by Rab27a in the ashen mouse, a model of Hermansky-Pudlak and Griscelli syndromes, is granule-specific and dependent on genetic background
The ashen (ash) mouse, a model for Hermansky-Pudlak syndrome (HPS) and for a subset of patients with Griscelli syndrome, presents with hypopigmentation, prolonged bleeding times, and platelet storage pool deficiency due to a mutation which abrogates expression of the Rab27a protein. Platelets of mice with the ashen mutation on the C3H/HeSnJ inbred strain background have greatly reduced amounts ...
متن کاملThe regulation of platelet-dense granules by Rab27a in the ashen mouse, a model of Hermansky-Pudlak and Griscelli syndromes, is granule-specific and dependent on genetic background.
The ashen (ash) mouse, a model for Hermansky-Pudlak syndrome (HPS) and for a subset of patients with Griscelli syndrome, presents with hypopigmentation, prolonged bleeding times, and platelet storage pool deficiency due to a mutation which abrogates expression of the Rab27a protein. Platelets of mice with the ashen mutation on the C3H/HeSnJ inbred strain background have greatly reduced amounts ...
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ژورنال
عنوان ژورنال: Journal of Cell Biology
سال: 2001
ISSN: 0021-9525,1540-8140
DOI: 10.1083/jcb.152.4.835