Morphological Aspects of Superoxide Dismutase - 1 Lateral Sclerosis and its Transgenic Mouse ModelMutation in Amyotrophic
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چکیده
// 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 ofMedicine, Hbrijo 272-J. Kumamoto 860-0811, ofPathology, Nbrthwestern Uhiversity Medical SchooL 3a3 East ChicagoAvenue, Chicngo ll] 60611-3e08, USA and tiCNSDtsec ses Researeh Unit, Pharmacia and b)ijohn inc., 301 Hlenrietta Stree4 Klxlamazoo, Afl 4900J. Usa
منابع مشابه
Protein disulphide isomerase protects against protein aggregation and is S-nitrosylated in amyotrophic lateral sclerosis.
Amyotrophic lateral sclerosis is a rapidly progressing fatal neurodegenerative disease characterized by the presence of protein inclusions within affected motor neurons. Endoplasmic reticulum stress leading to apoptosis was recently recognized to be an important process in the pathogenesis of sporadic human amyotrophic lateral sclerosis as well as in transgenic models of mutant superoxide dismu...
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Mutations in the Cu/Zn-superoxide dismutase (SOD-1) gene are responsible for a familial form of amyotrophic lateral sclerosis. In humans and experimental models, death of motor neurons is preceded by formation of cytoplasmic aggregates containing mutant SOD-1 protein. In our previous studies, heat shock protein 70 (HSP70) prolonged viability of cultured motor neurons expressing mutant human SOD...
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