Cerebral atrophy in amyotrophic lateral sclerosis parallels the pathological distribution of TDP43
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چکیده
منابع مشابه
Protein Aggregates in Pathological Inclusions of Amyotrophic Lateral Sclerosis
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disorder that is characterized by a progressive loss of upper and/or lower motor neurons (Bruijn et al., 2004). Dysfunction and death of these neurons lead to muscle weakness, atrophy and spasticity. A fatal event for the majority of patients is a failure of the respiratory muscles, which generally occurs within one to five years of dis...
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BACKGROUND Amyotrophic lateral sclerosis (ALS) is incurable and characterized by progressive paralysis of the muscles of the limbs, speech and swallowing, and respiration due to the progressive degeneration of voluntary motor neurons. Clinically indistinguishable ALS can be caused by genetic mutations of Cu/Zn superoxide dismutase (SOD1), TAR-DNA binding protein 43 (TDP43), or fused in sarcoma/...
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F rontotemporal dementia (FTD) with amyotrophic lateral sclerosis (ALS) presents with impaired language or behavior and declining motor function. Frontotemporal lobar degeneration with ubiquinated transactivating responsive sequence DNAbinding protein (TDP-43) inclusions is found postmortem in the affected brain areas of patients with ALS, FTD/ ALS, and many patients with FTD. Prior magnetic re...
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ژورنال
عنوان ژورنال: Brain Communications
سال: 2020
ISSN: 2632-1297
DOI: 10.1093/braincomms/fcaa061