Changes in Purkinje cell firing and gene expression precede behavioral pathology in a mouse model of SCA2
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چکیده
منابع مشابه
Changes in Purkinje cell firing and gene expression precede behavioral pathology in a mouse model of SCA2
Spinocerebellar ataxia type 2 (SCA2) is an autosomal dominantly inherited disorder, which is caused by a pathological expansion of a polyglutamine (polyQ) tract in the coding region of the ATXN2 gene. Like other ataxias, SCA2 most overtly affects Purkinje cells (PCs) in the cerebellum. Using a transgenic mouse model expressing a full-length ATXN2(Q127)-complementary DNA under control of the Pcp...
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Cerebellar Purkinje cells (PCs) are primarily affected in many spinocerebellar ataxias (SCA). In this study we investigated functional activity of PCs in transgenic mouse model of SCA2, a polyglutamine neurodegenerative hereditary disorder. In our studies we used extracellular single-unit recording method to compare spontaneous activity of PCs in age-matched wild-type mice and SCA2-58Q transgen...
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15 صفحه اولPower Spectral Density Analysis of Purkinje Cell Tonic and Burst Firing Patterns From a Rat Model of Ataxia and Riluzole Treated
Introduction: Purkinje Cell (PC) output displays a complex firing pattern consisting of high frequency sodium spikes and low frequency calcium spikes, and disruption in this firing behavior may contribute to cerebellar ataxia. Riluzole, neuroprotective agent, has been demonstrated to have neuroprotective effects in cerebellar ataxia. Here, the spectral analysis of PCs firing in control, 3-acety...
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ژورنال
عنوان ژورنال: Human Molecular Genetics
سال: 2012
ISSN: 0964-6906,1460-2083
DOI: 10.1093/hmg/dds427