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Human motor units in microfluidic devices are impaired by FUS mutations and improved by HDAC6 inhibition.

Stem cell reports - 14 Sept 2021

Stoklund Dittlau Katarina, Krasnow Emily N, Fumagalli Laura, Vandoorne Tijs, Baatsen Pieter, Kerstens Axelle, Giacomazzi Giorgia, Pavie Benjamin, Rossaert Elisabeth, Beckers Jimmy, Sampaolesi Maurilio, Van Damme Philip, Van Den Bosch Ludo

Abstract excerpt

Neuromuscular junctions (NMJs) ensure communication between motor neurons (MNs) and muscle; however, in MN disorders, such as amyotrophic lateral sclerosis (ALS), NMJs degenerate resulting in muscle atrophy. The aim of this study was to establish a versatile and reproducible in vitro model of a human motor unit to investigate the effects of ALS-causing mutations. Therefore, we generated a co-culture of human...

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Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

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Identifiers

PMID: 33891869
DOI: 10.1016/j.stemcr.2021.03.029
PMCID: PMC8452598

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