Changes in Motor Unit Loss and Axonal Regeneration Rate in Sporadic and Familiar Amyotrophic Lateral Sclerosis (ALS) — Possible Different Pathogenetic Mechanisms?
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منابع مشابه
Differential Motor Neuron Impairment and Axonal Regeneration in Sporadic and Familiar Amyotrophic Lateral Sclerosis with SOD-1 Mutations: Lessons from Neurophysiology
UNLABELLED Amyotrophic Lateral Sclerosis (ALS) is a degenerative disorder of the motor system. About 10% of cases are familial and 20% of these families have point mutations in the Cu/Zn superoxide dismutase 1 (SOD-1) gene. SOD-1 catalyses the superoxide radical (O(-2)) into hydrogen peroxide and molecular oxygen. The clinical neurophysiology in ALS plays a fundamental role in differential diag...
متن کاملDifferences in Motor Unit Loss and Axonal Regeneration Rate between Sporadic and Familiar Amyotrophic Lateral Sclerosis: An Undervalued Field of Research?
Amyotrophic Lateral Sclerosis (ALS) is a clinically and genetically heterogeneous, late-onset, neurodegenerative disorder of the motor system [1]. Five to ten percent of cases are familial and about 20% of these cases have point mutations in the Cu/Zn superoxide dismutase 1 (SOD-1) gene. Since its discovery, mutations in Cu/Zn superoxide dismutase (SOD-1) have stimulated a huge amount of intere...
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Objective(s): Amyotrophic lateral sclerosis (ALS), a fatal progressive neurodegenerative disorder, is the most common motor neuron disease in European populations. Approximately 10% of ALS cases are familial (FALS) and the other patients are considered as sporadic ALS (SALS). Among many ALS causing genes that have been identified, mutations in SOD1 and C9orf72 are the most common genetic causes...
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Amyotrophic lateral sclerosis (ALS) is a rapidly progressive and ultimately fatal neurodegenerative disease, caused by the loss of motor neurons in the brain and spinal cord. Although 10% of ALS cases are familial (FALS), the majority are sporadic (SALS) and probably associated to a multifactorial etiology. Currently there is no cure or prevention for ALS. A prerequisite to formulating therapeu...
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تاریخ انتشار 2017