Converging mechanisms of p53 activation drive motor neuron degeneration in spinal muscular atrophy

The hallmark of spinal muscular atrophy (SMA) – an inherited disease caused by ubiquitous deficiency in the SMN protein – is the selective degeneration of subsets of spinal motor neurons. Here we show that cell-autonomous activation of p53 occurs in vulnerable but not resistant motor neurons of SMA...

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Detalhes bibliográficos
Publicado no:Cell Rep
Main Authors: Simon, Christian M., Dai, Ya, Van Alstyne, Meaghan, Koutsioumpa, Charalampia, Pagiazitis, John G., Chalif, Joshua I., Wang, Xiaojian, Rabinowitz, Joseph E., Henderson, Christopher E., Pellizzoni, Livio, Mentis, George Z.
Formato: Artigo
Idioma:Inglês
Publicado em: 2017
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Article
Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC5747328/
https://ncbi.nlm.nih.gov/pubmed/29281826
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1016/j.celrep.2017.12.003
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