title: Subcellular organization of UBE3A in neurons.
abstract: Ubiquitination regulates a broad array of cellular processes, and defective ubiquitination is implicated in several neurological disorders. Loss of the E3 ubiquitin-protein ligase UBE3A causes Angelman syndrome. Despite its clinical importance, the normal role of UBE3A in neurons is still unclear. As a step toward deciphering its possible functions, we performed high-resolution light and electron microscopic immunocytochemistry. We report a broad distribution of UBE3A in neurons, highlighted by concentrations in axon terminals and euchromatin-rich nuclear domains. Our findings suggest that UBE3A may act locally to regulate individual synapses while also mediating global, neuronwide influences through the regulation of gene transcription. J. Comp. Neurol. 525:233-251, 2017. © 2016 Wiley Periodicals, Inc.
authors.affiliation: Department of Cell Biology and Physiology, University of North Carolina, Chapel Hill, North Carolina, 27599.
authors.forename: Alain C
authors.initials: AC
authors.lastname: Burette
authors.affiliation: Department of Cell Biology and Physiology, Neuroscience Center, and Carolina Institute for Developmental Disabilities, University of North Carolina, Chapel Hill, North Carolina, 27599.
authors.forename: Matthew C
authors.initials: MC
authors.lastname: Judson
authors.affiliation: Department of Cell Biology and Physiology, University of North Carolina, Chapel Hill, North Carolina, 27599.
authors.forename: Susan
authors.initials: S
authors.lastname: Burette
authors.affiliation: Department of Cell Biology and Physiology, University of North Carolina, Chapel Hill, North Carolina, 27599.
authors.forename: Kristen D
authors.initials: KD
authors.lastname: Phend
authors.affiliation: Department of Cell Biology and Physiology, Neurobiology Curriculum, Neuroscience Center, and Carolina Institute for Developmental Disabilities, University of North Carolina, Chapel Hill, North Carolina, 27599.
authors.forename: Benjamin D
authors.initials: BD
authors.lastname: Philpot
authors.affiliation: Department of Cell Biology and Physiology and Neuroscience Center, University of North Carolina, Chapel Hill, North Carolina, 27599.
authors.forename: Richard J
authors.initials: RJ
authors.lastname: Weinberg
mesh_terms: D000818:Animals; D001921:Brain / Q000378:metabolism*; D007091:Image Processing, Computer-Assisted; D007150:Immunohistochemistry; D008297:Male; D051379:Mice; D008810:Mice, Inbred C57BL; D018345:Mice, Knockout; D046529:Microscopy, Electron, Transmission; D009474:Neurons / Q000378:metabolism*; D044767:Ubiquitin-Protein Ligases / Q000096:biosynthesis*
chemical_list: C502429:Ube3a protein, mouse; D044767:Ubiquitin-Protein Ligases
keywords: Angelman syndrome; E6-AP; RRID:AB_10740376; RRID:nif-0000-30467; axon terminal; euchromatin; heterochromatin; mitochondria; ubiquitin-proteasome pathway