KPNB1 modulates the Machado–Joseph disease protein ataxin-3 through activation of the mitochondrial protease CLPP

Machado–Joseph disease (MJD) is characterized by a pathological expansion of the polyglutamine (polyQ) tract within the ataxin-3 protein. Despite its primarily cytoplasmic localization, polyQ-expanded ataxin-3 accumulates in the nucleus and forms intranuclear aggregates in the affected neurons. Due...

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Detalles Bibliográficos
Autores principales: Abeditashi, Mahkameh, Weber, Jonasz Jeremiasz, Pereira Sena, Priscila, Velic, Ana, Kalimeri, Maria, Incebacak Eltemur, Rana Dilara, Schmidt, Jana, Hübener-Schmid, Jeannette, Hauser, Stefan, Macek, Boris, Riess, Olaf, Schmidt, Thorsten
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Springer International Publishing 2022
Materias:
Original Article
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9259533/
https://www.ncbi.nlm.nih.gov/pubmed/35794401
http://dx.doi.org/10.1007/s00018-022-04372-5

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