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Merck
  • iPSC-derived functional human neuromuscular junctions model the pathophysiology of neuromuscular diseases.

iPSC-derived functional human neuromuscular junctions model the pathophysiology of neuromuscular diseases.

JCI insight (2019-09-20)
Chuang-Yu Lin, Michiko Yoshida, Li-Tzu Li, Akihiro Ikenaka, Shiori Oshima, Kazuhiro Nakagawa, Hidetoshi Sakurai, Eriko Matsui, Tatsutoshi Nakahata, Megumu K Saito
초록

The control of voluntary skeletal muscle contraction relies on action potentials, which send signals from the motor neuron through the neuromuscular junction (NMJ). Although dysfunction of the NMJ causes various neuromuscular diseases, a reliable in vitro system for disease modeling is currently unavailable. Here, we present a potentially novel 2-step, self-organizing approach for generating in vitro human NMJs from human induced pluripotent stem cells. Our simple and robust approach results in a complex NMJ structure that includes functional connectivity, recapitulating in vivo synapse formation. We used these in vitro NMJs to model the pathological features of spinal muscular atrophy, revealing the developmental and functional defects of NMJ formation and NMJ-dependent muscular contraction. Our differentiation system is therefore useful for investigating and understanding the physiology and pathology of human NMJs.

MATERIALS
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제품 설명

Sigma-Aldrich
Anti-Neurofilament 160 kDa Antibody, clone NN18, clone NN18, Chemicon®, from mouse
Sigma-Aldrich
Anti-Choline Acetyltransferase Antibody, Chemicon®, from goat