Restrictive Lung Disease in the Cu/Zn Superoxide-Dismutase 1 G93A Amyotrophic Lateral Sclerosis Mouse Model
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چکیده
منابع مشابه
Superoxide dismutase in CSF from amyotrophic lateral sclerosis patients with and without CuZn-superoxide dismutase mutations.
Mutations in CuZn-superoxide dismutase (CuZn-SOD) have been linked to familial amyotrophic lateral sclerosis (ALS), and motor neurone death is caused by the gain of a toxic property of the mutant protein. Here we determined amounts, activity and molecular forms of CuZn-SOD in CSF from ALS patients carrying the D90A and other CuZn-SOD mutations and patients without such mutations. There were no ...
متن کاملSuperoxide dismutase and amyotrophic lateral sclerosis.
Cover illustration: Lumbar spinal cord section from an ALS patient with sclerosis of the corticospinal tract. Klüver–Barerra staining. ABSTRACT Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease affecting motor neurons in the spinal cord, brain stem and motor cortex, leading to paralysis, respiratory failure and death. In about 5% of ALS cases, the disease is associa...
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// iDepartment ofPathology, Ibkyo PVbmenls Adbdical Uitiversity, 1(itwada-cho 8-1, Shinjuku-ku, 7blp,o 162-866a 2Division ofNleuropathology. ILfontofore Mledical Centen IU East210th Stree4 Bronx, NYi0467-249a Usa. 3Department ofPediatrics, YkemanashiMedical Uitiversity, Shimokato 111a 7timahe-cho, Nitkakoma-gun. Yitmanashi 4ew-38os, 4Department ofBiochemistry, Klatmamoto Uhiversity Schoot ofMed...
متن کاملSuperoxide Dismutase and Oxidative Stress in Amyotrophic Lateral Sclerosis
Oxidative stress is defined as the imbalance between reactive species such as free radicals and oxidants and the antioxidant defenses. Free radicals are molecules with one or more unpaired electrons, while oxidants are molecules with a high potential for taking electrons from other molecules. The more recognized reactive species are the reactive oxygen species (ROS), which include oxygen and it...
متن کاملMitochondrial Superoxide Dismutase Activity in Amyotrophic Lateral Sclerosis
Background: Evidence suggests that mitochondrial dysfunction and oxidative stress may be involved in the pathogenesis of Amyotrophic Lateral Sclerosis (ALS). Some studies show the presence of altered anti-oxidative defence enzyme activity in the blood of ALS patients. It has also been demonstrated that a superoxide-dismutase-1 (SOD1) enzyme fraction is located in the mitochondria. Objective: To...
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ژورنال
عنوان ژورنال: American Journal of Respiratory Cell and Molecular Biology
سال: 2017
ISSN: 1044-1549,1535-4989
DOI: 10.1165/rcmb.2016-0258le