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Published on: July 16, 2014
Enhanced Nociception in Angelman Syndrome Model Mice
Eric S McCoy1, Bonnie Taylor-Blake1, Megumi Aita1
1Department of Cell Biology and Physiology and University of North Carolina Neuroscience Center.
Summary
Angelman syndrome (AS) involves loss of the maternal UBE3A gene. This study found UBE3A is expressed in peripheral sensory neurons, but AS-related pain enhancements stem from central, not peripheral, nervous system changes.
Area of Science:
- Neuroscience
- Genetics
- Developmental Biology
Background:
- Angelman syndrome (AS) is a severe neurodevelopmental disorder linked to the loss or mutation of the maternal UBE3A allele.
- The role of UBE3A expression in the peripheral nervous system (PNS) and its contribution to AS phenotypes remain largely unknown.
Purpose of the Study:
- To investigate maternal and paternal UBE3A expression in dorsal root ganglion (DRG) neurons.
- To assess the impact of maternal UBE3A loss on nociceptive responses in AS model mice.
Main Methods:
- Analysis of UBE3A and UBE3A-ATS expression in mouse DRG neurons.
- Single-cell RNA sequencing of DRG transcriptomes.
- Behavioral testing of AS model mice with global and conditional maternal UBE3A deletion.
Main Results:
- Maternal UBE3A is expressed in large-diameter DRG neurons, while small-diameter neurons show biallelic UBE3A expression.
- UBE3A-ATS, an antisense transcript, is present in large-diameter neurons but low in small-diameter neurons.
- Global maternal UBE3A deletion in AS mice enhanced nociception, but this was not observed with sensory neuron-specific deletion.
Conclusions:
- Enhanced nociceptive responses in AS model mice are primarily mediated by central nervous system mechanisms, not peripheral sensory neurons.
- These findings provide novel insights into the sensory processing deficits associated with Angelman syndrome.
