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A Stem Cell Model of the Motor Circuit Uncouples Motor Neuron Death from Hyperexcitability Induced by SMN Deficiency
In spinal muscular atrophy, a neurodegenerative disease caused by ubiquitous deficiency in the survival motor neuron (SMN) protein, sensory-motor synaptic dysfunction and...
ALS-associated mutant FUS induces selective motor neuron degeneration through toxic gain of function
Mutations in FUS cause amyotrophic lateral sclerosis (ALS), including some of the most aggressive, juvenile-onset forms of the disease. FUS loss-of-function and toxic gain-of...
Role of Primary Afferents in the Developmental Regulation of Motor Axon Synapse Numbers on Renshaw Cells
Motor function in mammalian species depends on the
maturation of spinal circuits formed by a large variety
of interneurons that regulate motoneuron firing and
ALS/FTD Mutation-Induced Phase Transition of FUS Liquid Droplets and Reversible Hydrogels into Irreversible Hydrogels Impairs RNP Granule Function
The mechanisms by which mutations in FUS and other RNA binding proteins cause ALS and FTD remain controversial. We propose a model in which low-complexity (LC) domains of FUS drive its...
The ALS Association and Target ALS are pleased to announce the launch of a new collaborative effort to expand the collection of biofluids from people with ALS, to be used for research to better...
Findings point to potential therapeutic targets
NEW YORK, NY (February 19, 2015)
The discovery of key mediators of neurodegenerative phenotypes, especially prior to neurons becoming...