Neuromuscular Junction-on-a-Chip for Amyotrophic Lateral Sclerosis Modeling

Methods Mol Biol. 2024:2736:139-150. doi: 10.1007/7651_2022_474.

Abstract

Amyotrophic lateral sclerosis (ALS) is a progressive and degenerative disorder of the nervous system that can significantly reduce the physical activity of patients at the end stages. As the field of disease pathophysiology has advanced in recent years, studies have looked at the role of neuromuscular junction's dysfunctionality in ALS. In the past years, various in vitro and in vivo models were developed to scrutinize the underlying mechanisms of the disease and investigate the effects of candidate drugs, but the application of the developed models faced many challenges. Hence, the attentions shifted to cutting-edge technologies such as the organ-on-a-chip, which can mimic the pathophysiology of the disease as a special biological platform using patient-derived cells in the integration of engineering sciences to expand researchers' perspectives on the disease. In addition, organ-on-a-chip technology can reduce some of the challenges of using other in vitro and in vivo models, which can pave the way for other discoveries and advances in this disease.

Keywords: Amyotrophic lateral sclerosis; NMJ; Neuromuscular junction; Organ on a chip; Spheroid.

MeSH terms

  • Amyotrophic Lateral Sclerosis*
  • Animals
  • Disease Models, Animal
  • Exercise
  • Humans
  • Lab-On-A-Chip Devices
  • Neuromuscular Junction / physiology