The Angelman Syndrome Protein Ube3a/E6AP Is Required for Golgi Acidification and Surface Protein Sialylation

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The Angelman syndrome protein Ube3a/E6AP is required for Golgi acidification and surface protein sialylation.

Angelman syndrome (AS) is a severe disorder of postnatal brain development caused by neuron-specific loss of the HECT (homologous to E6AP carboxy terminus) domain E3 ubiquitin ligase Ube3a/E6AP. The cellular role of Ube3a remains enigmatic despite recent descriptions of synaptic and behavioral deficits in AS mouse models. Although neuron-specific imprinting is thought to limit the disease to th...

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ژورنال

عنوان ژورنال: Journal of Neuroscience

سال: 2013

ISSN: 0270-6474,1529-2401

DOI: 10.1523/jneurosci.1930-11.2013