Nature Communications (Sep 2019)
Src inhibition attenuates polyglutamine-mediated neuromuscular degeneration in spinal and bulbar muscular atrophy
- Madoka Iida,
- Kentaro Sahashi,
- Naohide Kondo,
- Hideaki Nakatsuji,
- Genki Tohnai,
- Yutaka Tsutsumi,
- Seiya Noda,
- Ayuka Murakami,
- Kazunari Onodera,
- Yohei Okada,
- Masahiro Nakatochi,
- Yuka Tsukagoshi Okabe,
- Shinobu Shimizu,
- Masaaki Mizuno,
- Hiroaki Adachi,
- Hideyuki Okano,
- Gen Sobue,
- Masahisa Katsuno
Affiliations
- Madoka Iida
- Department of Neurology, Nagoya University Graduate School of Medicine
- Kentaro Sahashi
- Department of Neurology, Nagoya University Graduate School of Medicine
- Naohide Kondo
- Department of Neurology, Nagoya University Graduate School of Medicine
- Hideaki Nakatsuji
- Department of Neurology, Nagoya University Graduate School of Medicine
- Genki Tohnai
- Department of Neurology, Nagoya University Graduate School of Medicine
- Yutaka Tsutsumi
- Department of Neurology, Nagoya University Graduate School of Medicine
- Seiya Noda
- Department of Neurology, Nagoya University Graduate School of Medicine
- Ayuka Murakami
- Department of Neurology, Nagoya University Graduate School of Medicine
- Kazunari Onodera
- Department of Neurology, Nagoya University Graduate School of Medicine
- Yohei Okada
- Department of Neurology, Nagoya University Graduate School of Medicine
- Masahiro Nakatochi
- Department of Nursing, Nagoya University Graduate School of Medicine
- Yuka Tsukagoshi Okabe
- Department of Advanced Medicine, Nagoya University Hospital
- Shinobu Shimizu
- Department of Advanced Medicine, Nagoya University Hospital
- Masaaki Mizuno
- Department of Advanced Medicine, Nagoya University Hospital
- Hiroaki Adachi
- Department of Neurology, University of Occupational and Environmental Health School of Medicine
- Hideyuki Okano
- Department of Physiology, Keio University School of Medicine
- Gen Sobue
- Brain and Mind Research Center, Nagoya University
- Masahisa Katsuno
- Department of Neurology, Nagoya University Graduate School of Medicine
- DOI
- https://doi.org/10.1038/s41467-019-12282-7
- Journal volume & issue
-
Vol. 10,
no. 1
pp. 1 – 15
Abstract
Spinal and bulbar muscular atrophy is a neuromuscular disease caused by an expanded CAG repeat in the androgen receptor gene. Here the authors show that Src kinase signaling is activated in a mouse model of the disease, and that Src inhibition improves pathology and behavioral symptoms in mice.