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Assessing the requirements of prenatal UBE3A expression for rescue of behavioral phenotypes in a mouse model for Angelman syndrome

BACKGROUND: Angelman syndrome (AS) is a rare neurodevelopmental disorder caused by the loss of functional ubiquitin protein ligase E3A (UBE3A). In neurons, UBE3A expression is tightly regulated by a mechanism of imprinting which suppresses the expression of the paternal UBE3A allele. Promising treat...

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Detalles Bibliográficos
Autores principales: Sonzogni, Monica, Zhai, Peipei, Mientjes, Edwin J., van Woerden, Geeske M., Elgersma, Ype
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2020
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7501605/
https://www.ncbi.nlm.nih.gov/pubmed/32948244
http://dx.doi.org/10.1186/s13229-020-00376-9