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RNA exosome mutations in pontocerebellar hypoplasia alter ribosome biogenesis and p53 levels

The RNA exosome is a ubiquitously expressed complex of nine core proteins (EXOSC1-9) and associated nucleases responsible for RNA processing and degradation. Mutations in EXOSC3, EXOSC8, EXOSC9, and the exosome cofactor RBM7 cause pontocerebellar hypoplasia and motor neuronopathy. We investigated th...

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Detalles Bibliográficos
Autores principales:	Müller, Juliane S, Burns, David T, Griffin, Helen, Wells, Graeme R, Zendah, Romance A, Munro, Benjamin, Schneider, Claudia, Horvath, Rita
Formato:	Online Artículo Texto
Lenguaje:	English
Publicado:	Life Science Alliance LLC 2020
Materias:	Research Articles
Acceso en línea:	https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7295610/ https://www.ncbi.nlm.nih.gov/pubmed/32527837 http://dx.doi.org/10.26508/lsa.202000678

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