Medical Research Council - London Institute of Medical Sciences

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Amyotrophic lateral sclerosis/ motor neurone disease II Junction Box

A living 3D model of a neuromuscular junction allows study of nerve faults in motor neurone disease

18 November 2018

Junction Box

Millions of times a day we turn our thoughts into actions - at neuromuscular junctions where electrical signals from motor neurons feed into our muscles. Amyotrophic lateral sclerosis (ALS) gradually weakens these connections for thousands of people around the world – but here’s a vital step towards new treatments. Inside a sort of lab-in-a-box called a microfluidic device, a bundle of nerve cells (artificially-coloured green with nuclei in blue) is reaching out tiny finger-like neurites towards muscle cells (purple) – creating a living 3D model of a neuromuscular junction. The neurons are modified to be optogenetic – they respond to pulses of laser light by pulling at the muscle cells, revealing weaker forces in cells grown from ALS sufferers. The next job is to bathe the diseased cells in different combinations of drugs, looking for clues to restoring neuromuscular junctions to full strength, in the hope of treating ALS as well as other conditions affecting the nervous system.

Written by John Ankers

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