Article
Macrophage migration inhibitory factor as a chaperone inhibiting accumulation of misfolded SOD1.
Neuron - 8 Apr 2015
Israelson Adrian, Ditsworth Dara, Sun Shuying, Song SungWon, Liang Jason, Hruska-Plochan Marian, McAlonis-Downes Melissa, Abu-Hamad Salah, Zoltsman Guy, Shani Tom, Maldonado Marcus, Bui Anh, Navarro Michael, Zhou Huilin, Marsala Martin, Kaspar Brian K, Da Cruz Sandrine, Cleveland Don W
Abstract excerpt
Mutations in superoxide dismutase (SOD1) cause amyotrophic lateral sclerosis (ALS), a neurodegenerative disease characterized by loss of motor neurons and accompanied by accumulation of misfolded SOD1 onto the cytoplasmic faces of intracellular organelles, including mitochondria and the endoplasmic reticulum (ER). Using inhibition of misfolded SOD1 deposition onto mitochondria as an assay, a chaperone activity...
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