Purkinje neuron Ca(2+) influx reduction rescues ataxia in SCA28 model

Spinocerebellar ataxia type 28 (SCA28) is a neurodegenerative disease caused by mutations of the mitochondrial protease AFG3L2. The SCA28 mouse model, which is haploinsufficient for Afg3l2, exhibits a progressive decline in motor function and displays dark degeneration of Purkinje cells (PC-DCD) of...

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Publicat a:J Clin Invest
Autors principals: Maltecca, Francesca, Baseggio, Elisa, Consolato, Francesco, Mazza, Davide, Podini, Paola, Young, Samuel M., Drago, Ilaria, Bahr, Ben A., Puliti, Aldamaria, Codazzi, Franca, Quattrini, Angelo, Casari, Giorgio
Format: Artigo
Idioma:Inglês
Publicat: American Society for Clinical Investigation 2014
Matèries:
Research Article
Accés en línia:https://ncbi.nlm.nih.gov/pmc/articles/PMC4382234/
https://ncbi.nlm.nih.gov/pubmed/25485680
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1172/JCI74770
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