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Wozniak MA, Baker BM, Chen CS, et al. The emerin-binding transcription factor Lmo7 is regulated by association with p130Cas at focal adhesions. PeerJ. 2013;1:e134doi: 10.7717/peerj.134.
Wozniak, M. A., Baker, B. M., Chen, C. S., & Wilson, K. L. (2013). The emerin-binding transcription factor Lmo7 is regulated by association with p130Cas at focal adhesions. PeerJ, 1e134. https://doi.org/10.7717/peerj.134
Wozniak, Michele A, et al. "The emerin-binding transcription factor Lmo7 is regulated by association with p130Cas at focal adhesions." PeerJ vol. 1 (2013): e134. doi: https://doi.org/10.7717/peerj.134
Wozniak MA, Baker BM, Chen CS, Wilson KL. The emerin-binding transcription factor Lmo7 is regulated by association with p130Cas at focal adhesions. PeerJ. 2013 Aug 20;1:e134. doi: 10.7717/peerj.134. eCollection 2013. PMID: 24010014; PMCID: PMC3757464.
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PeerJ. 2013 Aug 20;1:e134. doi: 10.7717/peerj.134. eCollection 2013.

The emerin-binding transcription factor Lmo7 is regulated by association with p130Cas at focal adhesions.

PeerJ

Michele A Wozniak, Brendon M Baker, Christopher S Chen, Katherine L Wilson

Affiliations

  1. Department of Cell Biology, Johns Hopkins University School of Medicine , Baltimore, MD , USA ; Department of Bioengineering, University of Pennsylvania , Philadelphia, PA , USA.

PMID: 24010014 PMCID: PMC3757464 DOI: 10.7717/peerj.134

Abstract

Loss of function mutations in the nuclear inner membrane protein, emerin, cause X-linked Emery-Dreifuss muscular dystrophy (X-EDMD). X-EDMD is characterized by contractures of major tendons, skeletal muscle weakening and wasting, and cardiac conduction system defects. The transcription factor Lmo7 regulates muscle- and heart-relevant genes and is inhibited by binding to emerin, suggesting Lmo7 misregulation contributes to EDMD disease. Lmo7 associates with cell adhesions and shuttles between the plasma membrane and nucleus, but the regulation and biological consequences of this dual localization were unknown. We report endogenous Lmo7 also associates with focal adhesions in cells, and both co-localizes and co-immunoprecipitates with p130Cas, a key signaling component of focal adhesions. Lmo7 nuclear localization and transcriptional activity increased significantly in p130Cas-null MEFs, suggesting Lmo7 is negatively regulated by p130Cas-dependent association with focal adhesions. These results support EDMD models in which Lmo7 is a downstream mediator of integrin-dependent signaling that allows tendon cells and muscles to adapt to and withstand mechanical stress.

Keywords: Emerin; Emery-Dreifuss muscular dystrophy; Focal adhesions; LEM-domain; Laminopathy; Lmo7; Nuclear envelope; Nucleoskeleton; Tendon; p130Cas

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