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- W1604996988 endingPage "433" @default.
- W1604996988 startingPage "418" @default.
- W1604996988 abstract "The motor neuron diseases collectively are disorders in which degeneration of the upper and lower motor neurons, either separately or in combination, give rise to a progressive loss of muscle function. The most common variant of adult onset is amyotrophic lateral sclerosis, a complex multisystem disorder in which multiple biological processes are impaired. The disease is neuropathologically characterized by motor neuron degeneration that includes a variety of intraneuronal inclusions of proteinaceous material. Both microglia and astrocytes contribute to the pathology. In 30–60% of patients, features of a frontotemporal degeneration are observed. Between 8–10% of amyotrophic lateral sclerosis cases are familial, including mutations in SOD1, TDP-43, FUS/TLS, ANG and senataxin. There is increasing evidence that alterations in RNA metabolism are of pathogenic importance." @default.
- W1604996988 created "2016-06-24" @default.
- W1604996988 creator A5037250294 @default.
- W1604996988 creator A5041532402 @default.
- W1604996988 creator A5062588593 @default.
- W1604996988 creator A5087316938 @default.
- W1604996988 date "2011-09-02" @default.
- W1604996988 modified "2023-09-25" @default.
- W1604996988 title "Amyotrophic Lateral Sclerosis, Primary Lateral Sclerosis and Spinal Muscular Atrophy" @default.
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