ALS Compendium

Title: Misfolded mutant SOD1 directly inhibits VDAC1 conductance in a mouse model of inherited ALS

Year Published: 2010

Observation: Misfolded mtSOD1 interacts with VDAC1 in vivo in spinal cords of SOD1 rats. ADP transport in outer mitochondria level is reduced in spinal cord of mutant SOD1 rats.Reduced VDAC1 activity diminishes survival of mutant SOD1 mice by accelerating disease onset

Comment: Establish a direct link between misfolded mutant SOD1 and mitochondrial dysfunction in fALS

Citation: Israelson, A., Arbel, N., Da Cruz, S., Ilieva, H., Yamanaka, K., Shoshan-Barmatz, V., & Cleveland, D. W. (2010). Misfolded mutant SOD1 directly inhibits VDAC1 conductance in a mouse model of inherited ALS. Neuron, 67(4), 575–87. doi:10.1016/j.neuron.2010.07.019

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