Altered Trafficking of Membrane Proteins in Purkinje Cells of SCA1 Transgenic Mice
作者： Pamela J. Skinner , Cynthia A. Vierra-Green , H. Brent Clark , Huda Y. Zoghbi , Harry T. Orr
DOI: 10.1016/S0002-9440(10)61766-X
关键词: Cell biology 、 Purkinje cell 、 Purkinje fibers 、 Membrane protein 、 Cell membrane 、 Polyglutamine tract 、 Biology 、 Vacuole 、 Immunology 、 Cytoplasm 、 Spinocerebellar ataxia
摘要: Spinocerebellar ataxia type 1 (SCA1) is a neurodegenerative disease caused by the expression of mutant ataxin-1 that contains an expanded polyglutamine tract. Overexpression in Purkinje cells transgenic mice results progressive and cell pathology are very similar to those seen SCA1 patients. Two prominent aspects presence cytoplasmic vacuoles dendritic atrophy. We found seem originate as large invaginations outer membrane. The contained proteins from somatodendritic membrane, including mGluR1, GluRΔ1/Δ2, GluR2/3, protein kinase C (PKC) γ. Further examination PKCγ revealed its sequestration into was accompanied concurrent loss localization at membrane decreased detection Western blot analysis. In addition, were immunoreactive for components ubiquitin/proteasome degradative pathway. These findings present link between vacuole formation dendrites indicate altered trafficking PKCγ, part neuronal dysfunction mice.
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