کد مقاله کد نشریه سال انتشار مقاله انگلیسی نسخه تمام متن
5506421 1400294 2017 12 صفحه PDF دانلود رایگان
عنوان انگلیسی مقاله ISI
Striatal synaptic dysfunction and altered calcium regulation in Huntington disease
موضوعات مرتبط
علوم زیستی و بیوفناوری بیوشیمی، ژنتیک و زیست شناسی مولکولی زیست شیمی
پیش نمایش صفحه اول مقاله
Striatal synaptic dysfunction and altered calcium regulation in Huntington disease
چکیده انگلیسی
Synaptic dysfunction and altered calcium homeostasis in the brain is common to many neurodegenerative disorders. Among these, Huntington disease (HD), which is inherited in an autosomal dominant fashion, can serve as a model for investigating these mechanisms. HD generally manifests in middle age as a disorder of movement, mood and cognition. An expanded polymorphic CAG repeat in the HTT gene results in progressive neurodegeneration that impacts striatal spiny projection neurons (SPNs) earliest and most severely. Striatal SPNs receive massive glutamatergic input from cortex and thalamus, and these excitatory synapses are a focus for early changes that can trigger aberrant downstream signaling to disrupt synaptic plasticity and lead to later degeneration. Mitochondrial dysfunction and altered intracellular calcium-induced calcium release and sequestration mechanisms add to the impairments in circuit function that may underlie prodromal cognitive and subtle motor deficits. These mechanisms and implications for developing disease-modifying therapy will be reviewed here.
ناشر
Database: Elsevier - ScienceDirect (ساینس دایرکت)
Journal: Biochemical and Biophysical Research Communications - Volume 483, Issue 4, 19 February 2017, Pages 1051-1062
نویسندگان
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