IGF1 neuronal response in the absence of MECP2 is dependent on TRalpha 3

IGF1 neuronal response in the absence of MECP2 is dependent on TRalpha 3

Author de Souza, Janaina S. Autor UNIFESP Google Scholar
Carromeu, Cassiano Google Scholar
Torres, Laila B. Google Scholar
Araujo, Bruno H. S. Autor UNIFESP Google Scholar
Cugola, Fernanda R. Google Scholar
Maciel, Rui M. B. Autor UNIFESP Google Scholar
Muotri, Alysson R. Google Scholar
Giannocco, Gisele Autor UNIFESP Google Scholar
Abstract Rett syndrome (RTT) is an X-linked neurodevelopmental disorder in which the MECP2 (methyl CpG-binding protein 2) gene is mutated. Recent studies showed that RTT-derived neurons have many cellular deficits when compared to control, such as: less synapses, lower dendritic arborization and reduced spine density. Interestingly, treatment of RTT-derived neurons with Insulin-like Growth Factor 1 (IGF1) could rescue some of these cellular phenotypes. Given the critical role of IGF1 during neurodevelopment, the present study used human induced pluripotent stem cells (iPSCs) from RTT and control individuals to investigate the gene expression profile of IGF1 and IGF1R on different developmental stages of differentiation. We found that the thyroid hormone receptor (TRalpha 3) has a differential expression profile. Thyroid hormone is critical for normal brain development. Our results showed that there is a possible link between IGF1/IGF1R and the TRalpha 3 and that over expression of IGF1R in RTT cells may be the cause of neurites improvement in neural RTT-derived neurons.
xmlui.dri2xhtml.METS-1.0.item-coverage Oxford
Language English
Sponsor California Institute for Regenerative Medicine (CIRM)
National Institutes of Health (NIH)
NARSAD Independent Investigator Grant
Coordenacao de Aperfeicoamento de Pessoal de Nivel Superior (CAPES, Brasilia, Brazil)
Fundacao de Amparo a Pesquisa do Estado de Sao Paulo (FAPESP, Sao Paulo, Brazil)
Grant number CIRM: TR2-01814, TR4-06747
NIH: R01MH094753
NIH: R01MH103134
NIH: U19MH107367
CAPES: 18952-12-7
FAPESP: 2014/08049-1
Date 2017
Published in Human Molecular Genetics. Oxford, v. 26, n. 2, p. 270-281, 2017.
ISSN 0964-6906 (Sherpa/Romeo, impact factor)
Publisher Oxford Univ Press
Extent 270-281
Origin http://dx.doi.org/10.1093/hmg/ddw384
Access rights Closed access
Type Article
Web of Science ID WOS:000397066400003
URI https://repositorio.unifesp.br/handle/11600/55244

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