Article
SQSTM1, a protective factor of SOD1-linked motor neuron disease, regulates the accumulation and distribution of ubiquitinated protein aggregates in neuron.
Neurochemistry international - 1 Sept 2022
Mitsui Shun, Otomo Asako, Sato Kai, Ishiyama Masahito, Shimakura Kento, Okada-Yamaguchi Chisa, Warabi Eiji, Yanagawa Toru, Aoki Masashi, Shang Hui-Fang, Hadano Shinji
Abstract excerpt
Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease characterized by selective loss of motor neurons in the brain and spinal cord. Recent studies have shown that mutations in SQSTM1 are linked to ALS. It has also been demonstrated that a systemic loss of SQSTM1 exacerbates disease phenotypes in an ALS mouse model. However, it is still unclear whether and how SQSTM1 in the central...
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