Cerebral cortical functional hyperconnectivity in a mouse model of spinocerebellar ataxia type 8 (SCA8).

• Tác giả: Russell E Carter, Timothy J Ebner, Morgan L Gerhart, Keerthi Manikonda, Angela K Nietz, Laurentiu S Popa, Laura P W Ranum
• Ngôn ngữ: eng
• Ký hiệu phân loại: 620.197 Organic fibrous materials
• Thông tin xuất bản: United States : Neurobiology of disease , 2025
• Bộ sưu tập: NCBI
• ID: 496744

Tóm tắt

Spinocerebellar Ataxia Type 8 (SCA8) is an inherited neurodegenerative disease caused by a bidirectionally expressed CTG•CAG expansion mutation in the ATXN8 and ATXN8OS genes. While SCA8 patients have motor abnormalities, patients may also exhibit psychiatric symptoms and cognitive dysfunction. It is difficult to elucidate how the disease alters brain function in areas with little or no degeneration producing both motor and cognitive symptoms. Using transparent polymer skulls and CNS-wide GCaMP6f expression, we studied neocortical networks throughout SCA8 progression using wide-field Ca