Article
Behavioral deficits in an Angelman syndrome model: effects of genetic background and age.
Behavioural brain research - 15 Apr 2013
Huang Hsien-Sung, Burns Andrew J, Nonneman Randal J, Baker Lorinda K, Riddick Natallia V, Nikolova Viktoriya D, Riday Thorfinn T, Yashiro Koji, Philpot Benjamin D, Moy Sheryl S
Abstract excerpt
Angelman syndrome (AS) is a severe neurodevelopmental disorder associated with disruption of maternally inherited UBE3A (ubiquitin protein ligase E3A) expression. At the present time, there is no effective treatment for AS. Mouse lines with loss of maternal Ube3a (Ube3a(m-/p+)) recapitulate multiple aspects of the clinical AS profile, including impaired motor coordination, learning deficits, and seizures. Thus,...
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