ETS transcription factor Erm controls subsynaptic gene expression in skeletal muscles

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.

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Journal Article | Published
Author
Hippenmeyer, SimonIST Austria ; Huber, Roland M; Ladle, David R; Murphy, Kenneth; Arber, Silvia
Abstract
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.
Publishing Year
Date Published
2007-09-06
Journal Title
Neuron
Volume
55
Issue
5
Page
726 - 740
IST-REx-ID

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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
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
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.
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.
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.
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.

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