Why is the expression level of BTBD10 in the ALS motor neuron declined?

2014 Fiscal Year Final Research Report

• Project/Area Number: 24790264
• Research Category: Grant-in-Aid for Young Scientists (B)
• Allocation Type: Multi-year Fund
• Research Field: General pharmacology
• Research Institution: Tokyo Medical University
• Principal Investigator: NAWA Mikiro 東京医科大学, 医学部, 助教 (10398620)
• Project Period (FY): 2012-04-01 – 2015-03-31
• Keywords: 筋萎縮性側索硬化症 / BTBD10

Outline of Final Research Achievements

Amyotrophic lateral sclerosis(ALS) is a fatal neurodegenerative disease with both upper and lower motor neuronal death. We previously reported that BTBD10, an Akt activator, suppressed ALS-related neuronal cell death. In this study, we focused on the role of BTBD10 in ALS and observed that reduction of expression level of BTBD10 using siRNA leads to caspase dependent cell death in motor neuronal cell line. We also observed neuronal cell death and a locomotive defect in C. elegans lacking BTBD10 gene. Compared with wild type littermates, the expression level of BTBD10 in the surviving motor neurons in spinal anterior horn was declined in G93A-superoxide dismutase 1 transgenic mice. We compared the number of BTBD10-positive motor neurons in spinal cord between sporadic ALS(SALS) patient and non-ALS control. We also observed the number of BTBD10-positive motor neuron was significantly decreased in the surviving motor neurons in the lamina 9 of SALS cases.

Free Research Field

分子病態薬理学