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In vivo Validation of Bimolecular Fluorescence Complementation (BiFC) to Investigate Aggregate Formation in Amyotrophic Lateral Sclerosis (ALS)

Mol Neurobiol. 2021-01; 
Emily K Don, Alina Maschirow, Rowan A W Radford, Natalie M Scherer, Andrés Vidal-Itriago, Alison Hogan, Cindy Maurel, Isabel Formella, Jack J Stoddart, Thomas E Hall, Albert Lee, Bingyang Shi, Nicholas J Cole, Angela S Laird, Andrew P Badrock, Roger S Chung, Marco Morsch
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摘要

Amyotrophic lateral sclerosis (ALS) is a form of motor neuron disease (MND) that is characterized by the progressive loss of motor neurons within the spinal cord, brainstem, and motor cortex. Although ALS clinically manifests as a heterogeneous disease, with varying disease onset and survival, a unifying feature is the presence of ubiquitinated cytoplasmic protein inclusion aggregates containing TDP-43. However, the precise mechanisms linking protein inclusions and aggregation to neuronal loss are currently poorly understood. Bimolecular fluorescence complementation (BiFC) takes advantage of the association of fluorophore fragments (non-fluorescent on their own) that are attached to an aggregation-prone protei... More

关键词

ALS, Aggregate formation, Bimolecular fluorescence complementation, FUS, TDP-43, Zebrafish