IMR Press / FBE / Volume 5 / Issue 3 / DOI: 10.2741/E683

Frontiers in Bioscience-Elite (FBE) is published by IMR Press from Volume 13 Issue 2 (2021). Previous articles were published by another publisher on a subscription basis, and they are hosted by IMR Press on as a courtesy and upon agreement with Frontiers in Bioscience.


Williams’ neural stem cells: new model for insight into microRNA dysregulation

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1 Laboratorio de Morfologia Celular y Molecular, Seccion de Estudios de Posgrado e Investigacion, Escuela Superior de Medicina, Instituto Politecnico Nacional, Plan de San Luis y Diaz Miron s/n, Col. Casco de Santo Tomas, Delegacion Miguel Hidalgo, C.P. 11340, Mexico, D. F.
2 Unidad de Investigacion Medica en Genetica Humana, Hospital de Pediatria, Centro Medico Nacional Siglo XXI, Instituto Mexicano del Seguro Social, Av Cuauhtemoc 330 Col. Doctores, Del. Cuauhtemoc, 06720 Mexico, D. F.
3 Unidad de Medicina Genomica, Hospital General de Mexico, Eje 2A Sur (Dr. Balmis) 148 Col. Doctores, Del. Cuauhtemoc, 06726, Mexico, D. F.
4 Departamento de Biologia del Desarrollo y Teratogenesis. Hospital Infantil de Mexico Federico Gomez, Doctor Marquez 162 Col. Doctores, Del. Cuauhtemoc, 06720 Mexico, D. F.
5 Laboratorio de Genomica del Cancer, Instituto Nacional de Medicina Genomica, Periferico Sur 4809 Col. Arenal Tepepan, Del. Tlalpan, 14610 Mexico, D. F.
6 Departamento de Seguimiento Pediatrico. Instituto Nacional de Perinatologia Dr. Isidro Espinosa de los Reyes, Prado Sur 800 Col. Lomas de Chapultepec, Del. Miguel Hidalgo, 11000, Mexico, D. F.
7 Departamento de Neurofarmacologia, Instituto Nacional de Psiquiatria Ramon de la Fuente Muniz, Calzada Mexico Xochimilco 101 Col. San Lorenzo Huipulco, Del. Tlalpan, 14370 Mexico, D. F.
8 Departamento de Morfologia, Facultad de Medicina Veterinaria y Zootecnia, Universidad Nacional Autonoma de Mexico, Circuito Exterior S/N Ciudad Universitaria, Del. Coyoacan, 04510, Mexico, D. F.

*Author to whom correspondence should be addressed.

Front. Biosci. (Elite Ed) 2013, 5(3), 1057–1073;
Published: 1 June 2013

Williams syndrome (WS) is a neurodevelopmental genetic disorder, due to a 7q11.23 hemizygous deletion. WS has a characteristic neurocognitive profile that includes intellectual disability (ID). Haploinsufficiency of some of the deleted genes is partially associated with the cognitive phenotype. The aim of this paper is to determine the differences in the microRNA (miRNA) expression in WS patients, using a neural cell model from the patient´s olfactory neuroepithelium (ONE), and to establish the relationship with those genes involved in neurodevelopment and neural function. To assess these goals, we made a comparative analysis of the miRNAs expression profile between WS patients and controls. Through an in silico analysis, we established potential pathways and targets associated with neural tissue. The expression profile shows 14 dysregulated miRNAs, including nervous system (NS)-rich miRNAs such as miR-125b, let-7c and miR-200. Most of these miRNAs have potential targets associated with NS functions while others have been reported to have specific neuronal functions. These data suggest that miRNAs widely contribute to the regulation of neurodevelopmental intrinsic processes, and that specific miRNAs could participate in WS neurobiology.

Williams syndrome
microRNA Expression
Neural Cell
Olfactory Neuroepithelim
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