کد مقاله کد نشریه سال انتشار مقاله انگلیسی نسخه تمام متن
5738632 1615045 2017 4 صفحه PDF دانلود رایگان
عنوان انگلیسی مقاله ISI
Research articleDisruption of GABA(A)-mediated intracortical inhibition in patients with chorea-acanthocytosis
موضوعات مرتبط
علوم زیستی و بیوفناوری علم عصب شناسی علوم اعصاب (عمومی)
پیش نمایش صفحه اول مقاله
Research articleDisruption of GABA(A)-mediated intracortical inhibition in patients with chorea-acanthocytosis
چکیده انگلیسی


- Ch-Ac patients display a striking impairment of GABA(A)-mediated intracortical inhibition.
- Motor cortex of Ch-Ac patients is disinhibited and hyperexcitable.
- Our results in humans support the existing data in mice models with this condition.

Chorea-acanthocytosis (Ch-Ac) is an autosomal recessive neurodegenerative disorder characterized by adult-onset chorea, acanthocytes in the peripheral blood, and Huntington's disease-like neuropsychiatric symptoms. Animal studies have shown mutation-related dysregulated cortical gamma-aminobutyric acid (GABA)ergic inhibitory networks in its pathophysiology. Herein we found that in patients with Ch-Ac there is a striking alteration of intracortical inhibitory circuits detected by using paired pulse transcranial magnetic stimulation protocols. Our findings show in vivo the functional disruption of GABA(A)-mediated networks in humans with Ch-Ac supporting the existing data in mice models with this condition.

ناشر
Database: Elsevier - ScienceDirect (ساینس دایرکت)
Journal: Neuroscience Letters - Volume 654, 27 July 2017, Pages 107-110
نویسندگان
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