RNA-Binding Proteins Associated Molecular Mechanisms of Motor Neuron Degeneration Pathogenesis
2014
Tang, Anna Y.
Motor neuron diseases are neurodegenerative disorders that trigger motor neurons to degenerate and lead to paralysis. Our understanding on the mechanisms of motor neuron degeneration has been enhanced by recent cellular and molecular researches. In this review, I highlight advances in RNA-binding proteins associated molecular mechanisms of motor neuron degeneration pathogenesis including ribonucleoprotein-associated motor neuron degeneration which is focused on RNA-binding protein aggregation associated aberrant RNA metabolism and stress granules mediated translational repression, neurofilament associated aggregate formation, and prion protein associated accumulation of misfolded proteins. Progress overviewed above will be valuable to the development of more targeted diagnostic tests and therapies.
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