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Cystatin B Deficiency Sensitizes Neurons to Oxidative Stress in Progressive Myoclonus Epilepsy, EPM1

The progressive myoclonus epilepsies, featuring the triad of myoclonus, seizures, and ataxia, comprise a large group of inherited neurodegenerative diseases that remain poorly understood and refractory to treatment. The Cystatin B gene is mutated in one of the most common forms of progressive myoclo...

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Autors principals: Lehtinen, Maria K., Tegelberg, Saara, Schipper, Hyman, Su, Haixiang, Zukor, Hillel, Manninen, Otto, Kopra, Outi, Joensuu, Tarja, Hakala, Paula, Bonni, Azad, Lehesjoki, Anna-Elina
Format: Artigo
Idioma:Inglês
Publicat: Society for Neuroscience 2009
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Accés en línia:https://ncbi.nlm.nih.gov/pmc/articles/PMC2694495/
https://ncbi.nlm.nih.gov/pubmed/19420257
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1523/JNEUROSCI.0682-09.2009
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