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

التفاصيل البيبلوغرافية
العنوان: The emerin-binding transcription factor Lmo7 is regulated by association with p130Cas at focal adhesions
المؤلفون: Michele A. Wozniak, Brendon M. Baker, Christopher S. Chen, Katherine L. Wilson
المصدر: PeerJ, Vol 1, p e134 (2013)
بيانات النشر: PeerJ Inc., 2013.
سنة النشر: 2013
المجموعة: LCC:Medicine
LCC:Biology (General)
مصطلحات موضوعية: Lmo7, p130Cas, Focal adhesions, Emery-Dreifuss muscular dystrophy, Laminopathy, LEM-domain, Medicine, Biology (General), QH301-705.5
الوصف: 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.
نوع الوثيقة: article
وصف الملف: electronic resource
اللغة: English
تدمد: 2167-8359
Relation: https://peerj.com/articles/134.pdf; https://peerj.com/articles/134/; https://doaj.org/toc/2167-8359
DOI: 10.7717/peerj.134
URL الوصول: https://doaj.org/article/762ad3e9002a4f34aec91e66f1c4c9e3
رقم الأكسشن: edsdoj.762ad3e9002a4f34aec91e66f1c4c9e3
قاعدة البيانات: Directory of Open Access Journals
الوصف
تدمد:21678359
DOI:10.7717/peerj.134