2024.12.13
Establishment of a novel amyotrophic lateral sclerosis patient (TARDBP N345K/+)-derived brain microvascular endothelial cell model reveals defective Wnt/β-catenin signaling: investigating diffusion barrier dysfunction and immune cell interaction.
A research team led by Assistant Professor Hideaki Nishihara of Yamaguchi University Graduate School of Medicine, in collaboration with Keio University and Tohoku University, has announced significant findings that could lead to treatments for amyotrophic lateral sclerosis (ALS).
In this study, the team established a cellular model of the blood-brain barrier (BBB) derived from induced pluripotent stem (iPS) cells obtained from a patient with familial ALS. Detailed analysis using this model revealed that the patient’s genetic background causes BBB dysfunction, increasing the risk of harmful substances entering the brain.
Furthermore, the study demonstrated that activating the “Wnt/β-catenin signaling pathway” can repair the BBB’s barrier function, thereby improving this abnormality. This model is expected to aid in the search for new therapeutic candidates for ALS.
These research findings were published in “Frontiers in Cell and Developmental Biology” on August 15, 2024.
- Division of Advanced Cell Therapy
- Hideaki Nishihara（Neurology）
- Paper Title：Establishment of a novel amyotrophic lateral sclerosis patient (TARDBP N345K/+)-derived brain microvascular endothelial cell model reveals defective Wnt/β-catenin signaling: investigating diffusion barrier dysfunction and immune cell interaction.
- Publication：Frontiers in Cell and Developmental Biology
- Author：Matsuo K et al.
- Publication Date: August 15, 2024
- DOI：https://doi.org/10.3389/fcell.2024.1357204
＜Research Inquiries＞
Department of Clinical Neurology Graduate School of Medicine, Yamaguchi University
Kinya Matsuo
Tel: 0836-22-2719
E-mail: matsuok@yamaguchi-u.ac.jp
＜Public Relations＞
Yamaguchi University The Research Institute for Cell Design and Medical Science (Life Science Support Section, Academic Research Division)
Tel: 0836-85-3065
E-mail: sh088@yamaguchi-u.ac.jp