Analysis of ion channel impairment in the pathogenesis of TTR amyloidosis

2017 Fiscal Year Final Research Report

• Project/Area Number: 16K19516
• Research Category: Grant-in-Aid for Young Scientists (B)
• Allocation Type: Multi-year Fund
• Research Field: Neurology
• Research Institution: Kumamoto University
• Principal Investigator: Misumi Yohei 熊本大学, 大学院生命科学研究部(医), 助教 (80625781)
• Project Period (FY): 2016-04-01 – 2018-03-31
• Keywords: アミロイドーシス / トランスサイレチン / イオンチャネル / 横細管

Outline of Final Research Achievements

Transthyretin (TTR) amyloidosis is a fatal systemic amyloidosis in which both wild type and mutant TTR form amyloid fibrils. Our pathological analysis revealed that TTR amyloid deposits were deposited mainly in the myocardial transverse tubule where ion channels are abundantly present. The amount of TTR amyloid deposition correlates with the decrease in calcium channel. Analysis using myocardial cell line and fluorescent calcium ion probe showed that TTR amyloid fibrils impairs calcium ion channel function. These data suggest that ion channel failure caused by TTR amyloid deposits is an important pathogenesis of TTR amyloidosis.

Free Research Field

アミロイドーシス