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Stem cell‐derived motoneurons: Tools for studying motoneuron disease and motoneuron development
Author(s) -
Rafuse Victor,
Soundararajan Praba,
Toma Jeremy
Publication year - 2012
Publication title -
the faseb journal
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.709
H-Index - 277
eISSN - 1530-6860
pISSN - 0892-6638
DOI - 10.1096/fasebj.26.1_supplement.207.4
Subject(s) - neuroscience , induced pluripotent stem cell , embryonic stem cell , biology , motor neuron , microbiology and biotechnology , sonic hedgehog , stem cell , myogenesis , neuromuscular junction , axon , myocyte , spinal cord , signal transduction , genetics , gene
Ten years ago, Tom Jessell and colleagues demonstrated for the first time that mouse embryonic stem (ES) cells can be directed to differentiate into motoneurons by simply treating them in culture with two mitogens, retinoic acid and a sonic hedgehog (Shh) agonist. Since this seminal discovery there has been an explosion of research using motoneurons derived from human and mouse ES cells, as well as induced pluripotent stem (iPS) cells, to study motoneuron disease and development. My presentation will first focus on developmental studies where we exploited the fact that mouse ES cell‐derived motoneurons differentiate into a single subclass of motoneurons that selectively innervate postural muscles lining the vertebral column. We show that ES cell‐derived motoneurons are an ideal model system to examine intracellular signaling cascades regulating motor axon guidance during embryogenesis. Second, I will describe a novel in vitro model system to study synapse formation at the neuromuscular junction using ES cell‐derived motoneurons and chick myotube co‐cultures. Finally, I will explore the use of motoneurons directly derived from fibroblasts as a tool to study motoneuron diseases.