Article
Mitochondrial dysfunction in CA1 hippocampal neurons of the UBE3A deficient mouse model for Angelman syndrome.
Neuroscience letters - 7 Jan 2011
Su Hailing, Fan Weiwei, Coskun Pinar E, Vesa Jouni, Gold June-Anne, Jiang Yong-Hui, Potluri Prasanth, Procaccio Vincent, Acab Allan, Weiss John H, Wallace Douglas C, Kimonis Virginia E
Abstract excerpt
Angelman syndrome (AS) is a severe neurological disorder caused by a deficiency of ubiquitin protein ligase E3A (UBE3A), but the pathophysiology of the disease remains unknown. We now report that in the brains of AS mice in which the maternal UBE3A allele is mutated (m-) and the paternal allele is potentially inactivated by imprinting (p+) (UBE3A m-\p+), the mitochondria are abnormal and exhibit a partial...
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