---
_id: '3144'
abstract:
- lang: eng
text: Accumulation of specific proteins at synaptic structures is essential for
synapse assembly and function, but mechanisms regulating local protein enrichment
remain poorly understood. At the neuromuscular junction (NMJ), subsynaptic nuclei
underlie motor axon terminals within extrafusal muscle fibers and are transcriptionally
distinct from neighboring nuclei. In this study, we show that expression of the
ETS transcription factor Erm is highly concentrated at subsynaptic nuclei, and
its mutation in mice leads to severe downregulation of many genes with normally
enriched subsynaptic expression. Erm mutant mice display an expansion of the muscle
central domain in which acetylcholine receptor (AChR) clusters accumulate, show
gradual fragmentation of AChR clusters, and exhibit symptoms of muscle weakness
mimicking congenital myasthenic syndrome (CMS). Together, our findings define
Erm as an upstream regulator of a transcriptional program selective to subsynaptic
nuclei at the NMJ and underscore the importance of transcriptional control of
local synaptic protein accumulation.
author:
- first_name: Simon
full_name: Simon Hippenmeyer
id: 37B36620-F248-11E8-B48F-1D18A9856A87
last_name: Hippenmeyer
orcid: 0000-0003-2279-1061
- first_name: Roland
full_name: Huber, Roland M
last_name: Huber
- first_name: David
full_name: Ladle, David R
last_name: Ladle
- first_name: Kenneth
full_name: Murphy, Kenneth
last_name: Murphy
- first_name: Silvia
full_name: Arber, Silvia
last_name: Arber
citation:
ama: Hippenmeyer S, Huber R, Ladle D, Murphy K, Arber S. ETS transcription factor
Erm controls subsynaptic gene expression in skeletal muscles. Neuron. 2007;55(5):726-740.
doi:10.1016/j.neuron.2007.07.028
apa: Hippenmeyer, S., Huber, R., Ladle, D., Murphy, K., & Arber, S. (2007).
ETS transcription factor Erm controls subsynaptic gene expression in skeletal
muscles. Neuron. Elsevier. https://doi.org/10.1016/j.neuron.2007.07.028
chicago: Hippenmeyer, Simon, Roland Huber, David Ladle, Kenneth Murphy, and Silvia
Arber. “ETS Transcription Factor Erm Controls Subsynaptic Gene Expression in Skeletal
Muscles.” Neuron. Elsevier, 2007. https://doi.org/10.1016/j.neuron.2007.07.028.
ieee: S. Hippenmeyer, R. Huber, D. Ladle, K. Murphy, and S. Arber, “ETS transcription
factor Erm controls subsynaptic gene expression in skeletal muscles,” Neuron,
vol. 55, no. 5. Elsevier, pp. 726–740, 2007.
ista: Hippenmeyer S, Huber R, Ladle D, Murphy K, Arber S. 2007. ETS transcription
factor Erm controls subsynaptic gene expression in skeletal muscles. Neuron. 55(5),
726–740.
mla: Hippenmeyer, Simon, et al. “ETS Transcription Factor Erm Controls Subsynaptic
Gene Expression in Skeletal Muscles.” Neuron, vol. 55, no. 5, Elsevier,
2007, pp. 726–40, doi:10.1016/j.neuron.2007.07.028.
short: S. Hippenmeyer, R. Huber, D. Ladle, K. Murphy, S. Arber, Neuron 55 (2007)
726–740.
date_created: 2018-12-11T12:01:39Z
date_published: 2007-09-06T00:00:00Z
date_updated: 2021-01-12T07:41:22Z
day: '06'
doi: 10.1016/j.neuron.2007.07.028
extern: 1
intvolume: ' 55'
issue: '5'
month: '09'
page: 726 - 740
publication: Neuron
publication_status: published
publisher: Elsevier
publist_id: '3551'
quality_controlled: 0
status: public
title: ETS transcription factor Erm controls subsynaptic gene expression in skeletal
muscles
type: journal_article
volume: 55
year: '2007'
...