Book Chapter

Genetics of motor neuron disorders: From gene diversity to common cellular conspirators in selective neuronal killing

RK Sheean, BJ Turner

Drosophila Melanogaster Models of Motor Neuron Disease | Published : 2013


Motor neuron disorders (MNDs) are a spectrum of progressive and incapacitating neurological diseases leading to motor impairment, disability and in most cases death. The clinical phenotype of MNDs depends on the relative degeneration of (a) upper and lower motor neurons and (b) their proximal cell bodies and distal axons. Despite limited progress in the search for effective therapies, there has been recent and explosive discovery of dozens of causative genes in MNDs. This arsenal of genetic evidence has illuminated key cellular pathways disrupted in motor neurons, axons and supporting ensheathing glial cells, providing fresh insights into determinants of neuronal susceptibility and loss in M..

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