Converging Mechanisms of p53 Activation Drive Motor Neuron Degeneration in Spinal Muscular Atrophy

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 m...

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Bibliographic Details
Main Authors: Christian M. Simon, Ya Dai, Meaghan Van Alstyne, Charalampia Koutsioumpa, John G. Pagiazitis, Joshua I. Chalif, Xiaojian Wang, Joseph E. Rabinowitz, Christopher E. Henderson, Livio Pellizzoni, George Z. Mentis
Format: Article
Language:English
Published: Elsevier 2017-12-01
Series:Cell Reports
Subjects:
neurodegenerative disease
spinal muscular atrophy
SMA
survival motor neuron
SMN
motor neurons
p53
phosphorylation
cell death
Online Access:http://www.sciencedirect.com/science/article/pii/S2211124717317990

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http://www.sciencedirect.com/science/article/pii/S2211124717317990

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