Article
Motor neuron-specific disruption of proteasomes, but not autophagy, replicates amyotrophic lateral sclerosis.
The Journal of biological chemistry - 14 Dec 2012
Tashiro Yoshitaka, Urushitani Makoto, Inoue Haruhisa, Koike Masato, Uchiyama Yasuo, Komatsu Masaaki, Tanaka Keiji, Yamazaki Maya, Abe Manabu, Misawa Hidemi, Sakimura Kenji, Ito Hidefumi, Takahashi Ryosuke
Abstract excerpt
Evidence suggests that protein misfolding is crucially involved in the pathogenesis of amyotrophic lateral sclerosis (ALS). However, controversy still exists regarding the involvement of proteasomes or autophagy in ALS due to previous conflicting results. Here, we show that impairment of the ubiquitin-proteasome system, but not the autophagy-lysosome system in motor neurons replicates ALS in mice. Conditional...
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