Authors: Fornetti E, Testa S, De, F, Fuoco C, Bernardini S, Pozo V, Stokin GB, Giannitelli SM, Rainer A, Bigot A, Zoccali C, Baldi J, Sandonà D, Rizzi R, Bearzi C, Forte G, Cannata S, and Gargioli C.
Advanced Materials Technologies, 2022.
Scientists use Axion’s bioelectronic methods to explore an in vitro model of the complex neuromuscular junction.
The highly complex neuromuscular junction (NMJ)—a chemical synapse between a motor neuron and a muscle fiber—is implicated in a number of diseases and disorders, but modeling the structure in vitro has been challenging for scientists. Using microfluidics and an organ-on-a-chip approach, researchers in this study coculture induced pluripotent stem cell (iPSC)-derived neural progenitors with muscle-derived human mesenchymal stem cells (hMSCs) to reproduce the human NMJ and examine the underlying mechanisms of alpha-sarcoglycanopathy, a type of limb-girdle muscular dystrophy with varying levels of severity.
To explore functionality of the hiPSC-derived motor neurons in vitro during the characterization phase of the experiment, scientists used Axion’s noninvasive, label-free Maestro platform and showed that the cultured neurons exhibit expected electrical activity and form networks. The scientists demonstrate that skeletal muscle plays a significant role in axonal outgrowth of motor neurons and NMJ formation while the motor neurons had little influence on skeletal muscle development. Their alpha-sarcoglycanopathy model was able to identify defects affecting NMJ formation and although additional research is needed, the authors suggest that advanced in vitro models may enable researchers to pursue new therapeutic strategies for neuromuscular diseases in the future
