Functional Connectivity under Optogenetic Control Allows Modeling of Human Neuromuscular Disease.

ABSTRACT: Capturing the full potential of human pluripotent stem cell (PSC)-derived neurons in disease modeling and regenerative medicine requires analysis in complex functional systems. Here we establish optogenetic control in human PSC-derived spinal motorneurons and show that co-culture of these cells with human myoblast-derived skeletal muscle builds a functional all-human neuromuscular junction that can be triggered to twitch upon light stimulation. To model neuromuscular disease we incubated these co-cultures with IgG from myasthenia gravis patients and active complement. Myasthenia gravis is an autoimmune disorder that selectively targets neuromuscular junctions. We saw a reversible reduction in the amplitude of muscle contractions, representing a surrogate marker for the characteristic loss

SUBMITTER: Steinbeck JA 

PROVIDER: S-EPMC4707991 | biostudies-literature | 2016 Jan

REPOSITORIES: biostudies-literature