Mutant copper-zinc superoxide dismutase (SOD1) induces protein secretion pathway alterations and exosome release in astrocytes: implications for disease spreading and motor neuron pathology in amyotrophic lateral sclerosis*
نویسندگان
چکیده
Dulbecco Telethon Institute at IRCCS Istituto di Ricerche Farmacologiche Mario Negri, Milano, Italy Department of Neuroscience, IRCCS Istituto di Ricerche Farmacologiche Mario Negri, Milano, Milano, Italy Bio-imaging Unit, Department of Cardiovascular Research, IRCCS Istituto di Ricerche Farmacologiche Mario Negri, Milano, Milano, Italy Current address: Burke Medical Research Institute, Weill Medical College, Cornell University, NY These authors contributed equally to this work
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SOD1 misplacing and mitochondrial dysfunction in amyotrophic lateral sclerosis pathogenesis
Amyotrophic lateral sclerosis (ALS) is a fatal motor neuron disease presenting as sporadic (sALS) or familial (fALS) forms. Even if the list of the genes underlining ALS greatly expanded, defects in superoxide dismutase 1 (SOD1), encoding the copper/zinc SOD1, still remain a major cause of fALS and are likely involved also in apparently sporadic presentations. The pathogenesis of ALS is still u...
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