Jianxun Yi

Dec 4, 2024 | biorxiv.org | Ang Li |Li Dong |Xuejun Li |Jianxun Yi

AbstractDifferent muscles exhibit varied susceptibility to degeneration in Amyotrophic Lateral Sclerosis (ALS), a fatal neuromuscular disorder. Extraocular muscles (EOMs) are particularly resistant to ALS progression and exploring the underlying molecular nature may deliver great therapeutic value. Reactive aldehyde 4-hydroxynonenal (HNE) is implicated in ALS pathogenesis and ALDH3A1 is an inactivation-resistant intracellular detoxifier of 4-HNE protecting eyes against UV-induced oxidative stress.

Apr 25, 2024 | elifesciences.org | Ang Li |Jianxun Yi |Xuejun Li |Li Dong

eLife assessment The manuscript by Jingsong Zhou and colleagues uncovers why the extraocular muscles (EOMs) are preserved while other muscles undergo degenerative changes in amyotrophic lateral sclerosis (ALS). In this work, the authors have used a mouse model of familial ALS that carries a G93A mutation in the Sod1 gene to demonstrate that NaBu treatment partially restores the integrity of NMJ in the limb and diaphragm muscles of G93A mice.

Sep 24, 2023 | biorxiv.org | Ang Li |Jianxun Yi |Xuejun Li |Li Dong

AbstractAmyotrophic lateral sclerosis (ALS) is a fatal neuromuscular disorder characterized by progressive weakness of almost all skeletal muscles, whereas extraocular muscles (EOMs) are comparatively spared.

Feb 13, 2023 | biorxiv.org | Ang Li |Jianxun Yi |Xuejun Li |Li Dong

AbstractAmyotrophic lateral sclerosis (ALS) is a fatal neuromuscular disease that affects all voluntary muscles in the body, leading to paralysis and respiratory failure, while the extraocular muscles (EOMs) are largely spared even at the end-stage of ALS.