Functional NMJ system for personalized ALS modeling and drug testing
2020
University of Central Florida, Orlando, USA
Loss of the neuromuscular junction (NMJ) is an early and critical hallmark in all forms of amyotrophic lateral sclerosis (ALS). An ALS-NMJ system is established by using motor neuron stem cells from ALS patients and integrating them into a chambered system. Myotube contractions are recorded while MNs are stimulated by field electrodes and a set of clinically relevant parameters are defined to characterize NMJ function. The utilization of three ALS mutant lines revealed significant NMJ deficits in all mutant lines, but variations in severity and parameter selection, as well as in response to drug treatment, highlight the need for patient-specific models.
A human‐based functional NMJ system for personalized ALS modeling and drug testing
James J. Hickman
Added on: 09-24-2020
[1] https://onlinelibrary.wiley.com/doi/10.1002/adtp.202000133?emci=489a5dd4-bceb-ea11-8b03-00155d0394bb&emdi=0f2124dd-63ec-ea11-8b03-00155d0394bb&ceid=2015591
