Subcellular Organization of UBE3A in Neurons

被引：39

作者：

Burette, Alain C. ^{[1
]}

Judson, Matthew C. ^{[2
,3
]}

Burette, Susan ^{[1
]}

Phend, Kristen D. ^{[1
]}

Philpot, Benjamin D. ^{[3
,4
]}

Weinberg, Richard J. ^{[1
,5
]}

机构：

[1] Univ N Carolina, Dept Cell Biol & Physiol, Chapel Hill, NC 27599 USA

[2] Univ N Carolina, Dept Cell Biol & Physiol, Neurosci Ctr, Chapel Hill, NC 27599 USA

[3] Univ N Carolina, Carolina Inst Dev Disabil, Chapel Hill, NC 27599 USA

[4] Univ N Carolina, Dept Cell Biol & Physiol, Neurobiol Curriculum, Neurosci Ctr, Chapel Hill, NC 27599 USA

[5] Univ N Carolina, Neurosci Ctr, Chapel Hill, NC 27599 USA

来源：

JOURNAL OF COMPARATIVE NEUROLOGY | 2017年 / 525卷 / 02期

关键词：

Angelman syndrome; E6-AP; ubiquitin-proteasome pathway; axon terminal; mitochondria; euchromatin; heterochromatin; RRID:nif-0000-30467; RRID:AB_10740376; SYNDROME PROTEIN UBE3A; ANGELMAN-SYNDROME; MOUSE MODEL; MEDIATED DEGRADATION; UBIQUITIN LIGASE; GENE UBE3A; AUTISM; PROTEASOME; ALPHA; E6-AP;

D O I：

10.1002/cne.24063

中图分类号：

Q189 [神经科学];

学科分类号：

071006 ;

摘要：

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. (C) 2016 Wiley Periodicals, Inc.

引用

页码：233 / 251

页数：19

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