TY - JOUR
T1 - Williams' neural stem cells
T2 - New model for insight into microRNA dysregulation
AU - Palacios-Reyes, Carmen
AU - Espinosa, Ana
AU - Contreras, Alejandra
AU - Ordonez, Rosa
AU - Hidalgo-Miranda, Alfredo
AU - Rubio-Gayosso, Ivan
AU - Garcia-Alonso, Patricia
AU - Benitez-King, Gloria
AU - Ramirez-Rodriguez, Gerardo
AU - Najera, Nayelli
AU - Ita-Islas, Israel
AU - Araujo, Antonieta
AU - Romero-Cordoba, Sandra
AU - Palma, Icela
PY - 2013/6/1
Y1 - 2013/6/1
N2 - 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.
AB - 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.
KW - MicroRNA expression
KW - Neural cell
KW - Olfactory neuroepithelim
KW - Williams syndrome
UR - http://www.scopus.com/inward/record.url?scp=84884306008&partnerID=8YFLogxK
M3 - Artículo
SN - 1945-0494
VL - 5 E
SP - 1057
EP - 1073
JO - Frontiers in Bioscience - Elite
JF - Frontiers in Bioscience - Elite
IS - 3
ER -