ALS/FTD-linked mutation in FUS suppresses intraaxonal protein synthesis and drives disease without nuclear loss-of-function of FUS

Lopez-Erauskin J, Tadokoro T, Baughn MW, Myers B, McAlonis-Downes M, Chillon-Marinas C,  Asiaban JN, Artates J, Bui AT, Vetto AP, Lee SK, Vy Le A, Sun Y,  Jambeau M, Boubaker J, Swing D, Qiu J, Hicks GG, Ouyang Z, Fu XD, Tessarollo L, Ling SC, Parone PA,  Shaw CE, Marsala M, Lagier-Tourenne C, Cleveland DW, Da Cruz S.

Neuron, 2018

Summary:

Follow researchers as they use Maestro MEA technology to help demonstrate that human ALS/FTD-linked mutations in FUS induce a gain of toxicity that includes stress-mediated suppression in intra-axonal translation, synaptic dysfuntion, and progressive age-dependent motor and cognitive disease.

Keywords: ALS, amyotrophic lateral sclerosis, FTD, FUS, frontotemporal dementia, neurodegeneration, synaptic activity, motor neuron