Determination of abnormal prion protein in peripheral organs of various prion diseases

2019 Fiscal Year Final Research Report

• Project/Area Number: 17K07097
• Research Category: Grant-in-Aid for Scientific Research (C)
• Allocation Type: Multi-year Fund
• Section: 一般
• Research Field: Nerve anatomy/Neuropathology
• Research Institution: Kyushu University
• Principal Investigator: Honda Hiroyuki 九州大学, 医学研究院, 助教 (90624057)
• Project Period (FY): 2017-04-01 – 2020-03-31
• Keywords: プリオン蛋白 / 下垂体 / 内分泌

Outline of Final Research Achievements

Immunohisatochemistry for prion protein (PrP) on some prion diseases (sporadic Creutzfeldt-Jakob diseases and Gerstmann-Strussler-Scheinker diseases) revealed positive findings of PrP in in many adenohypophyseal cells. Proteomics analysis also revealed PrP in pituitary samples. Western blots showed excessive glycosylation was attached to pituitary PrP, and protease-resistant PrP (PrPres) was also detected. In addition, PrP deposition was also identified histologically in the islets of Langerhans, skin, muscle and peripheral nerves. It was suggested that PrP is involved in human endocrine mechanism, and it was also shown that pituitary tissue of prion disease cases contains PrPres and may be a source of infection.

Free Research Field

神経病理学

Academic Significance and Societal Importance of the Research Achievements

ヒトの下垂体前葉細胞の細胞質に、多くのプリオン蛋白が含まれており、それらが通常のプリオン蛋白と異なり過剰な糖鎖修飾をされていた。また、プリオン病症例の下垂体組織にはプロテアーゼ抵抗性プリオン蛋白が含まれている事も同時に示した。ヒトの内分泌機構にプリオン蛋白が関与しており、またプリオン病症例の下垂体組織は感染源になりうる可能性がある事を示した。