Cell Death Differ.
The ALS-linked E102Q mutation in Sigma receptor-1 leads to ER stress-mediated defects in protein homeostasis and dysregulation of RNA-binding proteins.
Dreser, A., Vollrath, J.T., Sechi, A., Johann, S., Roos, A., Yamoah, A., Katona, I., Bohlega, S., Wiemuth, D., Tian, Y., Schmidt, A., Vervoorts, J., Dohmen, M., Beyer, C., Anink, J., Aronica, E., Troost, D., Weis, J., and Goswami, A.
Notes: These authors studied the role of a mutation in the endoplasmic reticulum chaperone Sigma receptor-1 (E102Q) in ALS pathology. As part of their analysis, they used the Mitochondrial ToxGlo™ Assay to assess the effects of the mutation on mitochondrial function. MCF-7 cells transfected with various constructs were first evaluated for loss of membrane integrity using a fluorescence-based cell viability assay. The Mitochondrial ToxGlo™ reagent was then added to the same plate and the luminescent readout used to detect ATP as a measure of mitochondrial function. (4956)
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