The mechanisms underlying the encephalopathy of inherited glycosylphosphatidy linositol deficiency(IGD) disease at synapse, neuron and neural circuit levels

• Project/Area Number: 25K10298
• Research Category: Grant-in-Aid for Scientific Research (C)
• Allocation Type: Multi-year Fund
• Section: 一般
• Review Section: Basic Section 49030:Experimental pathology-related
• Research Institution: University of Tsukuba
• Principal Investigator: 張 王其 筑波大学, 人間系, 客員研究員 (20525604)
• Project Period (FY): 2025-04-01 – 2028-03-31
• Project Status: Granted (Fiscal Year 2025)
• Budget Amount: ¥4,680,000 (Direct Cost: ¥3,600,000、Indirect Cost: ¥1,080,000); Fiscal Year 2027: ¥1,300,000 (Direct Cost: ¥1,000,000、Indirect Cost: ¥300,000); Fiscal Year 2026: ¥1,950,000 (Direct Cost: ¥1,500,000、Indirect Cost: ¥450,000); Fiscal Year 2025: ¥1,430,000 (Direct Cost: ¥1,100,000、Indirect Cost: ¥330,000)
• Keywords: GPI / IGD / epilepsy / gene therapy / sugar protein

Outline of Research at the Start

In current work, we will apply state-of-the-art histology, electrophysiology, single-cell transcriptomics analysis and behavioral analysis to thoroughly investigate the pathological changes at the synapse, cell, and neural circuit levels of IGD model mice. In addition, AAV mediated circuit-specific rescue experiment will be applied to confirm the neural circuits crucially involved in the pathophysiology.