--- _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' ...