Amyotrophic Lateral Sclerosis (ALS)-on-a-chip
2023
University of Central Florida, Research Parkway, USA
Amyotrophic Lateral Sclerosis (ALS) is a neurodegenerative disease that leads to death within 2–5 years of diagnosis. In this study, it was investigated how improving of human skeletal muscle (hSKM) viability and function affects the integrity of neuromuscular junctions (NMJ) in ALS.
Therefore, an ALS NMJ human-on-a-chip model was developed using patient-derived induced pluripotent stem cells (iPSCs) differentiated into motoneurons and myoblasts. The model was used to study the effect of hSKM-specific creatine treatment on clinically relevant functional ALS NMJ parameters. Results indicated comparatively enhanced NMJ numbers, and stability, as well as reduced fatigue index, across creatine-treated systems.
The study demonstrates the utilization of organ-on-a-chip technology for recapitulating complex human diseases like ALS and its applicability for drug toxicological and efficacy screening.
The effect of skeletal muscle-specific creatine treatment on ALS NMJ integrity and function
James J. Hickman
Added on: 10-16-2023
[1] https://www.mdpi.com/1422-0067/24/17/13519
