| Citation | Gonzalez-Aguilera C, Ikegami K, Ayuso C, de Luis A, Iniguez M, Cabello J, Lieb JD, Askjaer P. Genome-wide analysis links emerin to neuromuscular junction activity in Caenorhabditis elegans. Genome Biol, 2014. |
| PubMed ID | 24490688 |
| Short Description | Genome-wide analysis links emerin to neuromuscular junction activity in Caenorhabditis elegans. GEO Record: GSE44682 Platform: GPL13776 Download gene-centric, log2 transformed data: WBPaper00044786.ce.rs.csv |
| # of Conditions | 9 |
Full Description
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BACKGROUND: Laminopathies are diseases characterized by defects in nuclear envelope structure. A well-known example is Emery-Dreifuss muscular dystrophy, which is caused by mutations in the human lamin A/C and emerin genes. While most nuclear envelope proteins are ubiquitously expressed, laminopathies often affect only a subset of tissues. The molecular mechanisms underlying these tissue-specific manifestations remain elusive. We hypothesize that different functional subclasses of genes might be differentially affected by defects in specific nuclear envelope components. RESULTS: Here we determine genome-wide DNA association profiles of two nuclear envelope components, lamin/LMN-1 and emerin/EMR-1 in adult Caenorhabditis elegans. Although both proteins bind to transcriptionally inactive regions of the genome, EMR-1 is enriched at genes involved in muscle and neuronal function. Deletion of either EMR-1 or LEM-2, another integral envelope protein, causes local changes in nuclear architecture as evidenced by altered association between DNA and LMN-1. Transcriptome analyses reveal that EMR-1 and LEM-2 are associated with gene repression, particularly of genes implicated in muscle and nervous system function. We demonstrate that emr-1, but not lem-2, mutants are sensitive to the cholinesterase inhibitor aldicarb, indicating altered activity at neuromuscular junctions. CONCLUSIONS: We identify a class of elements that bind EMR-1 but do not associate with LMN-1, and these are enriched for muscle and neuronal genes. Our data support a redundant function of EMR-1 and LEM-2 in chromatin anchoring to the nuclear envelope and gene repression. We demonstrate a specific role of EMR-1 in neuromuscular junction activity that may contribute to Emery-Dreifuss muscular dystrophy in humans. Experimental Details: RNASeq.elegans.WBStrain00000001.WBls:0000002.Hermaphrodite.WBbt:0007833.SRP018867.SRX245821 RNASeq.elegans.WBStrain00000001.WBls:0000002.Hermaphrodite.WBbt:0007833.SRP018867.SRX245822 RNASeq.elegans.WBStrain00000001.WBls:0000002.Hermaphrodite.WBbt:0007833.SRP018867.SRX245823 RNASeq.elegans.WBStrain00003835.WBls:0000002.Hermaphrodite.WBbt:0007833.SRP018867.SRX245824 RNASeq.elegans.WBStrain00003835.WBls:0000002.Hermaphrodite.WBbt:0007833.SRP018867.SRX245825 RNASeq.elegans.WBStrain00003834.WBls:0000002.Hermaphrodite.WBbt:0007833.SRP018867.SRX245826 RNASeq.elegans.WBStrain00003834.WBls:0000002.Hermaphrodite.WBbt:0007833.SRP018867.SRX245827 RNASeq.elegans.WBStrain00003834.WBls:0000002.Hermaphrodite.WBbt:0007833.SRP018867.SRX245828 RNASeq.elegans.WBStrain00003834.WBls:0000002.Hermaphrodite.WBbt:0007833.SRP018867.SRX245829. |
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