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SETBP1 accumulation induces P53 inhibition and genotoxic stress in neural progenitors underlying neurodegeneration in Schinzel-Giedion syndrome
The investigation of genetic forms of juvenile neurodegeneration could shed light on the causative mechanisms of neuronal loss. Schinzel-Giedion syndrome (SGS) is a fatal developmental syndrome caused by mutations in the SETBP1 gene, inducing the accumulation of its protein product. SGS features mul...
Autores principales: | Banfi, Federica, Rubio, Alicia, Zaghi, Mattia, Massimino, Luca, Fagnocchi, Giulia, Bellini, Edoardo, Luoni, Mirko, Cancellieri, Cinzia, Bagliani, Anna, Di Resta, Chiara, Maffezzini, Camilla, Ianielli, Angelo, Ferrari, Maurizio, Piazza, Rocco, Mologni, Luca, Broccoli, Vania, Sessa, Alessandro |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Nature Publishing Group UK
2021
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8245514/ https://www.ncbi.nlm.nih.gov/pubmed/34193871 http://dx.doi.org/10.1038/s41467-021-24391-3 |
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