Unravelling a mechanistic link between mitophagy defect, mitochondrial malfunction, and apoptotic neurodegeneration in Mucopolysaccharidosis VII.

• Tác giả: Apurba Das, Rupak Datta, Nishan Mandal
• Ngôn ngữ: eng
• Ký hiệu phân loại: 025.3177 Bibliographic analysis and control
• Thông tin xuất bản: United States : Neurobiology of disease , 2025
• Bộ sưu tập: NCBI
• ID: 497484

Tóm tắt

Cognitive disability and neurodegeneration are prominent symptoms of Mucopolysaccharidosis VII (MPS VII), a lysosomal storage disorder caused by β-glucuronidase enzyme deficiency. Yet, the mechanism of neurodegeneration in MPS VII remains unclear thereby limiting the scope of targeted therapy. We aimed to bridge this knowledge gap by employing the β-glucuronidase-deficient (CG2135