Article
Amyotrophic lateral sclerosis-related mutant superoxide dismutase 1 aggregates inhibit 14-3-3-mediated cell survival by sequestration into the JUNQ compartment.
Human molecular genetics - 15 Sept 2017
Park Ju-Hwang, Jang Hae Rim, Lee In Young, Oh Hye Kyung, Choi Eui-Ju, Rhim Hyangshuk, Kang Seongman
Abstract excerpt
Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disorder characterized by motor neuron loss in the spinal cord and brain. Mutations in the superoxide dismutase 1 (SOD1) gene have been linked to familial ALS. To elucidate the role of SOD1 mutations in ALS, we investigated 14-3-3, a crucial regulator of cell death that was identified in patients with familial ALS. In a transgenic mouse model...
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