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Cellosaurus publication CLPUB00461

Publication number CLPUB00461
Authors Goigoreva E.V., Valetdinova K.R., Ustyantseva E.I., Shevchenko A.I., Medvedev S.P., Mazurok N.A., Maretina M.A., Kuranova M.L., Kiselev A.V., Baranov V.S., Zakyan S.M.
Title Neural differentiation of patient-specific induced pluripotent stem cells from patients with a hereditary form of spinal muscular atrophy.
Citation Genes and Cells 11:2.70-2.79(2016)
Web pages https://www.researchgate.net/publication/323187310_Neural_differentiation_of_patient-specific_induced_pluripotent_stem_cells_from_patients_with_a_hereditary_form_of_spinal_muscular_atrophy
Abstract Induced pluripotent stem cells (iPSCs) give the possibility for disease modeling, drug and toxicology screening and development of the new therapeutic approaches. Directed differentiation of iPSCs into specialized cell types represents a unique tool in order to study and model certain diseases, which affects specific type of cells, in vitro. one of the typical example of such diseases is spinal muscular atrophy, which is caused by mutations in the SMN1 gene (survival motor neuron 1 gene), leading to selective death of motor neurons. Patient-specific iPSCs were derived from the patient with a hereditary form of spinal muscular atrophy I type and expressed the markers of pluripotency (NANOG, TRA-1-60, SSEA4, OCT4, KLF4, MYC, REX1, and others). Spontaneous differentiation of the obtained cells resulted in the appearance of derivatives of the three germ layers: ecto-, meso-and endoderm. neural differentiation showed the appearance of the early neural markers (PAX6, SOX2, NESTIN, tuJ1, PSA- NCAM), the late mature neural markers (MAP2, NF200, GFAP), and the mature motor neurons' markers (ISL1 and CHAT). neurons derived from patient- specific iPSCs are perspective model for studying the features of the cells, which are altered in spinal muscular atrophy.
Cell lines CVCL_UF65; ICGi005-A
CVCL_UF66; ICGi005-B