Analyzing the ER stress response in ALS patient derived motor neurons identifies druggable neuroprotective targets
Ontology highlight
ABSTRACT:
INSTRUMENT(S): Orbitrap Fusion Lumos
ORGANISM(S): Homo Sapiens (ncbitaxon:9606)
SUBMITTER: 
Alban Ordureau
PROVIDER: MSV000093190 | MassIVE | Wed Oct 25 08:34:00 BST 2023
REPOSITORIES: MassIVE
Publications
Analyzing the ER stress response in ALS patient derived motor neurons identifies druggable neuroprotective targets.
Watts Michelle E ME Giadone Richard M RM Ordureau Alban A Holton Kristina M KM Harper J Wade JW Rubin Lee L LL
Frontiers in cellular neuroscience 20240119
Amyotrophic lateral sclerosis (ALS) is a degenerative motor neuron (MN) disease with severely limited treatment options. Identification of effective treatments has been limited in part by the lack of predictive animal models for complex human disorders. Here, we utilized pharmacologic ER stressors to exacerbate underlying sensitivities conferred by ALS patient genetics in induced pluripotent stem cell (iPSC)-derived motor neurons (MNs). In doing so, we found that thapsigargin and tunicamycin exp ...[more]
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