title: Modeling ALS with motor neurons derived from human induced pluripotent stem cells.
abstract: Directing the differentiation of induced pluripotent stem cells into motor neurons has allowed investigators to develop new models of amyotrophic lateral sclerosis (ALS). However, techniques vary between laboratories and the cells do not appear to mature into fully functional adult motor neurons. Here we discuss common developmental principles of both lower and upper motor neuron development that have led to specific derivation techniques. We then suggest how these motor neurons may be matured further either through direct expression or administration of specific factors or coculture approaches with other tissues. Ultimately, through a greater understanding of motor neuron biology, it will be possible to establish more reliable models of ALS. These in turn will have a greater chance of validating new drugs that may be effective for the disease.
authors.affiliation: Board of Governors Regenerative Medicine Institute, Cedars-Sinai Medical Center, Los Angeles, California, USA.
authors.forename: Samuel
authors.initials: S
authors.lastname: Sances
authors.affiliation: ALS Association Headquarters, Washington, DC, USA.
authors.forename: Lucie I
authors.initials: LI
authors.lastname: Bruijn
authors.affiliation: Centre for Clinical Brain Sciences, University of Edinburgh, Edinburgh, UK.
authors.forename: Siddharthan
authors.initials: S
authors.lastname: Chandran
authors.affiliation: Harvard Stem Cell Institute, Harvard University, Cambridge, Massachusetts, USA.
authors.forename: Kevin
authors.initials: K
authors.lastname: Eggan
authors.affiliation: Board of Governors Regenerative Medicine Institute, Cedars-Sinai Medical Center, Los Angeles, California, USA.
authors.forename: Ritchie
authors.initials: R
authors.lastname: Ho
authors.affiliation: Department of Stem Cell and Regenerative Biology, Harvard University, Cambridge, Massachusetts, USA.
authors.forename: Joseph R
authors.initials: JR
authors.lastname: Klim
authors.affiliation: Centre for Clinical Brain Sciences, University of Edinburgh, Edinburgh, UK.|Centre for Integrative Physiology, University of Edinburgh, UK.
authors.forename: Matt R
authors.initials: MR
authors.lastname: Livesey
authors.affiliation: Department of Pathology and Cell Biology, Columbia University, New York, New York, USA.
authors.forename: Emily
authors.initials: E
authors.lastname: Lowry
authors.affiliation: Harvard Stem Cell Institute, Harvard University, Cambridge, Massachusetts, USA.|Department of Stem Cell and Regenerative Biology, Harvard University, Cambridge, Massachusetts, USA.|Center for Brain Science, Harvard University, Cambridge, Massachusetts, USA.
authors.forename: Jeffrey D
authors.initials: JD
authors.lastname: Macklis
authors.affiliation: Board of Governors Regenerative Medicine Institute, Cedars-Sinai Medical Center, Los Angeles, California, USA.
authors.forename: David
authors.initials: D
authors.lastname: Rushton
authors.affiliation: Harvard Stem Cell Institute, Harvard University, Cambridge, Massachusetts, USA.|Department of Stem Cell and Regenerative Biology, Harvard University, Cambridge, Massachusetts, USA.|Center for Brain Science, Harvard University, Cambridge, Massachusetts, USA.
authors.forename: Cameron
authors.initials: C
authors.lastname: Sadegh
authors.affiliation: Board of Governors Regenerative Medicine Institute, Cedars-Sinai Medical Center, Los Angeles, California, USA.
authors.forename: Dhruv
authors.initials: D
authors.lastname: Sareen
authors.affiliation: Department of Pathology and Cell Biology, Columbia University, New York, New York, USA.
authors.forename: Hynek
authors.identifier: http://orcid.org/0000-0002-7817-0080
authors.initials: H
authors.lastname: Wichterle
authors.affiliation: Waisman Center, University of Wisconsin, Madison, Wisconsin, USA.
authors.forename: Su-Chun
authors.initials: SC
authors.lastname: Zhang
authors.affiliation: Board of Governors Regenerative Medicine Institute, Cedars-Sinai Medical Center, Los Angeles, California, USA.
authors.forename: Clive N
authors.initials: CN
authors.lastname: Svendsen
mesh_terms: D000690:Amyotrophic Lateral Sclerosis / Q000473:pathology*; D000818:Animals; D002454:Cell Differentiation / Q000502:physiology; D002478:Cells, Cultured; D018920:Coculture Techniques / Q000379:methods; D006801:Humans; D057026:Induced Pluripotent Stem Cells / Q000473:pathology* / Q000502:physiology; D009046:Motor Neurons / Q000473:pathology* / Q000502:physiology