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PMID:17785180
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| Citation |
Hippenmeyer, S, Huber, RM, Ladle, DR, Murphy, K and Arber, S (2007) ETS transcription factor Erm controls subsynaptic gene expression in skeletal muscles. Neuron 55:726-40 |
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| 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. |
| Links |
PubMed Online version:10.1016/j.neuron.2007.07.028 |
| Keywords |
Animals; Cell Nucleus/genetics; Cell Nucleus/metabolism; DNA-Binding Proteins/genetics; DNA-Binding Proteins/metabolism; Down-Regulation/genetics; Gene Expression Regulation, Developmental/genetics; Mice; Mice, Knockout; Muscle Weakness/genetics; Muscle Weakness/metabolism; Muscle Weakness/physiopathology; Muscle, Skeletal/growth & development; Muscle, Skeletal/metabolism; Muscle, Skeletal/physiopathology; Mutation/genetics; Myasthenia Gravis/genetics; Myasthenia Gravis/metabolism; Myasthenia Gravis/physiopathology; Neuromuscular Junction/genetics; Neuromuscular Junction/growth & development; Neuromuscular Junction/metabolism; Receptor Aggregation/genetics; Receptors, Nicotinic/genetics; Receptors, Nicotinic/metabolism; Synaptic Membranes/genetics; Synaptic Membranes/metabolism; Synaptic Transmission/genetics; Transcription Factors/genetics; Transcription Factors/metabolism; Transcriptional Activation/genetics |
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