title: Williams' neural stem cells: new model for insight into microRNA dysregulation.
abstract: 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 patients 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.
authors.affiliation: 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, Mexico.
authors.forename: Carmen
authors.initials: C
authors.lastname: Palacios-Reyes
authors.forename: Ana
authors.initials: A
authors.lastname: Espinosa
authors.forename: Alejandra
authors.initials: A
authors.lastname: Contreras
authors.forename: Rosa
authors.initials: R
authors.lastname: Ordonez
authors.forename: Alfredo
authors.initials: A
authors.lastname: Hidalgo-Miranda
authors.forename: Ivan
authors.initials: I
authors.lastname: Rubio-Gayosso
authors.forename: Patricia
authors.initials: P
authors.lastname: Garcia-Alonso
authors.forename: Gloria
authors.initials: G
authors.lastname: Benitez-King
authors.forename: Gerardo
authors.initials: G
authors.lastname: Ramirez-Rodriguez
authors.forename: Nayelli
authors.initials: N
authors.lastname: Najera
authors.forename: Israel
authors.initials: I
authors.lastname: Ita-Islas
authors.forename: Antonieta
authors.initials: A
authors.lastname: Araujo
authors.forename: Sandra
authors.initials: S
authors.lastname: Romero-Cordoba
authors.forename: Icela
authors.initials: I
authors.lastname: Palma
mesh_terms: D000293:Adolescent; D000328:Adult; D001483:Base Sequence; D016022:Case-Control Studies; D002648:Child; D017931:DNA Primers; D005260:Female; D006801:Humans; D035683:MicroRNAs / Q000235:genetics / Q000502:physiology*; D008954:Models, Biological; D058953:Neural Stem Cells / Q000166:cytology*; D016133:Polymerase Chain Reaction; D018980:Williams Syndrome / Q000473:pathology*
chemical_list: D017931:DNA Primers; D035683:MicroRNAs