A role for motoneuron subtype–selective ER stress in disease manifestations of FALS mice

By Smita Saxena, E. Cabuy, P. Caroni. Nature Neuroscience, 2009

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Non–cell autonomous toxicity in neurodegenerative disorders: ALS and beyond (2009, background): “Similar deletion from motor neurons and interneurons (by a Lhx3-driven Cre transgene) primarily delayed disease onset and early disease progression (Wang et al., 2009).”
Endoplasmic Reticulum Stress and Oxidative Stress in Cell Fate Decision and Human Disease (2014, background): “In a mouse model of familial ALS, salubrinal reduced the accumulation of poly-ubiquitinated protein inside motor neurons, improved motor neuron and muscle function, and prolonged survival (146).”
Autophagy and apoptosis dysfunction in neurodegenerative disorders. (2014, positive): “Strong evidence obtained from a longitudinal analysis proposed that different responses to ER stress in subpopulations of motor neurons affect the manifestation and progression of ALS (Saxena et al., 2009).”
Oxidative stress in ALS: key role in motor neuron injury and therapeutic target. (2010, background): “presymptomatic ages in three different mutant SOD1 transgenic mouse models [132].”
Integrating the mechanisms of apoptosis induced by endoplasmic reticulum stress (2011, background): “and at certain doses can promote survival in certain cell culture and mouse disease models associated with prolonged ER stres”