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Dis Model Mech 7:799-809.","DOI":"10.1242\/dmm.015719"},{"key":"key-10.4103\/1673-5374.206635-63","doi-asserted-by":"crossref","unstructured":"Pepper JP, Wang TV, Hennes V, Sun SY, Ichida JK (2016) Human induced pluripotent stem cell-derived motor neuron transplant for neuromuscular atrophy in a mouse model of sciatic nerve injury. JAMA Facial Plast Surg doi:10.1001\/jamafacial.2016.1544.","DOI":"10.1001\/jamafacial.2016.1544"},{"key":"key-10.4103\/1673-5374.206635-64","doi-asserted-by":"crossref","unstructured":"Philippidou P, Walsh CM, Aubin J, Jeannotte L, Dasen JS (2012) Sustained Hox5 gene activity is required for respiratory motor neuron development. 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Sci Transl Med 5:208ra149.","DOI":"10.1126\/scitranslmed.3007529"},{"key":"key-10.4103\/1673-5374.206635-70","doi-asserted-by":"crossref","unstructured":"Schmid A, DiDonato CJ (2007) Animal models of spinal muscular atrophy. J Child Neurol 22:1004-1012.","DOI":"10.1177\/0883073807305667"},{"key":"key-10.4103\/1673-5374.206635-71","doi-asserted-by":"crossref","unstructured":"Son MJ, Kwon Y, Son MY, Seol B, Choi HS, Ryu SW, Choi C, Cho YS (2015) Mitofusins deficiency elicits mitochondrial metabolic reprogramming to pluripotency. Cell Death Differ 22:1957-1969.","DOI":"10.1038\/cdd.2015.43"},{"key":"key-10.4103\/1673-5374.206635-72","doi-asserted-by":"crossref","unstructured":"Stadtfeld M, Nagaya M, Utikal J, Weir G, Hochedlinger K (2008) Induced pluripotent stem cells generated without viral integration. Science 322:945-949.","DOI":"10.1126\/science.1162494"},{"key":"key-10.4103\/1673-5374.206635-73","doi-asserted-by":"crossref","unstructured":"Su H, Wang L, Cai J, Yuan Q, Yang X, Yao X, Wong WM, Huang W, Li Z, Wan JB, Wang Y, Pei D, So KF, Qin D, Wu W (2013) Transplanted motoneurons derived from human induced pluripotent stem cells form functional connections with target muscle. Stem Cell Res 11:529-539.","DOI":"10.1016\/j.scr.2013.02.007"},{"key":"key-10.4103\/1673-5374.206635-74","doi-asserted-by":"crossref","unstructured":"Takahashi K, Yamanaka S (2006) Induction of pluripotent stem cells from mouse embryonic and adult fibroblast cultures by defined factors. Cell 126:663-676.","DOI":"10.1016\/j.cell.2006.07.024"},{"key":"key-10.4103\/1673-5374.206635-75","unstructured":"Vaskova EA, Stekleneva AE, Medvedev SP, Zakian SM (2013) \u201cEpigenetic memory\u201d phenomenon in induced pluripotent stem cells. Acta Naturae 5:15-21."},{"key":"key-10.4103\/1673-5374.206635-76","doi-asserted-by":"crossref","unstructured":"Wainger BJ, Kiskinis E, Mellin C, Wiskow O, Han SS, Sandoe J, Perez NP, Williams LA, Lee S, Boulting G, Berry JD, Brown RH, Jr., Cudkowicz ME, Bean BP, Eggan K, Woolf CJ (2014) Intrinsic membrane hyperexcitability of amyotrophic lateral sclerosis patient-derived motor neurons. Cell Rep 7:1-11.","DOI":"10.1016\/j.celrep.2014.03.019"},{"key":"key-10.4103\/1673-5374.206635-77","doi-asserted-by":"crossref","unstructured":"Wang G, McCain ML, Yang L, He A, Pasqualini FS, Agarwal A, Yuan H, Jiang D, Zhang D, Zangi L, Geva J, Roberts AE, Ma Q, Ding J, Chen J, Wang DZ, Li K, Wang J, Wanders RJ, Kulik W, et al. (2014) Modeling the mitochondrial cardiomyopathy of Barth syndrome with induced pluripotent stem cell and heart-on-chip technologies. Nat Med 20:616-623.","DOI":"10.1038\/nm.3545"},{"key":"key-10.4103\/1673-5374.206635-78","doi-asserted-by":"crossref","unstructured":"Wijesekera LC, Leigh PN (2009) Amyotrophic lateral sclerosis. Orphanet J Rare Dis 4:3.","DOI":"10.1186\/1750-1172-4-3"},{"key":"key-10.4103\/1673-5374.206635-79","doi-asserted-by":"crossref","unstructured":"Wong PC, Pardo CA, Borchelt DR, Lee MK, Copeland NG, Jenkins NA, Sisodia SS, Cleveland DW, Price DL (1995) An adverse property of a familial ALS-linked SOD1 mutation causes motor neuron disease characterized by vacuolar degeneration of mitochondria. Neuron 14:1105-1116.","DOI":"10.1016\/0896-6273(95)90259-7"},{"key":"key-10.4103\/1673-5374.206635-80","doi-asserted-by":"crossref","unstructured":"Xu CC, Denton KR, Wang ZB, Zhang X, Li XJ (2016) Abnormal mitochondrial transport and morphology as early pathological changes in human models of spinal muscular atrophy. Dis Models Mech 9:39-49.","DOI":"10.1242\/dmm.021766"},{"key":"key-10.4103\/1673-5374.206635-81","doi-asserted-by":"crossref","unstructured":"Yoo AS, Sun AX, Li L, Shcheglovitov A, Portmann T, Li Y, Lee-Messer C, Dolmetsch RE, Tsien RW, Crabtree GR (2011) MicroRNA-mediated conversion of human fibroblasts to neurons. Nature 476:228-231.","DOI":"10.1038\/nature10323"},{"key":"key-10.4103\/1673-5374.206635-82","doi-asserted-by":"crossref","unstructured":"Zhou H, Wu S, Joo JY, Zhu S, Han DW, Lin T, Trauger S, Bien G, Yao S, Zhu Y, Siuzdak G, Scholer HR, Duan L, Ding S (2009) Generation of induced pluripotent stem cells using recombinant proteins. 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