Innovation and Development

Function of Ube3a in Polarized Dendrite Development

On Jan. 2, 2013, Dr. Xiong Zhiqi’s research group at the Shanghai Institutes for Biological Sciences, published a paper entitled “The Angelman syndrome protein Ube3a is required for polarized dendrite morphogenesis in pyramidal neurons” in the Journal of Neuroscience. This work was carried out by graduate student Miao Sheng and his colleagues under the supervision of Dr. Xiong Zhiqi. Angelman syndrome is a severe neurodevelopmental disorder caused by deficient Ube3A gene expression and characterized by severe intellectual and developmental disability, sleep disturbance, seizures, jerky movements etc.  In this study, the group found a novel function of Ube3a in specifying the polarization of pyramidal neuron dendritic arbors in mice, and identified Ube3a cytoplasm-localized isoform 2 as an important regulatory molecule for Golgi-dependent control of normal polarized dendrite development. Impairment of dendrite polarity in pyramidal neurons caused by Ube3a depletion may lead to alterations in neural circuitry that underlie behavioral and cognitive dysfunctions associated with Angelman syndrome.