Human in vitro neuromuscular junction model to functionally dissect the pathogenic mechanism of anti-AChR autoantibody-positive myasthenia gravis.

ABSTRACT:

Background

Myasthenia gravis is a rare autoimmune disease mediated by autoantibodies directed against acetylcholine receptors (AChRs) at the neuromuscular junction. These autoantibodies cause dysfunction through AChR blockade, AChR degradation due to crosslinking and internalisation, and complement activation.

Methods

A novel in vitro model of the human neuromuscular junction was established on a microfluidic platform to investigate the effect of anti-AChR autoantibodies on complement activation and neuromuscular transmission and the mechanism of action of complement inhibition in myasthenia gravis. The NeuroMuscle^TM platform enabled the connection of human induced pluripotent stem-cell-derived motor neuron spheroids with three-dimensional cultures of skeletal mus

SUBMITTER: Shin B 

PROVIDER: S-EPMC12817423 | biostudies-literature | 2025 Dec

REPOSITORIES: biostudies-literature