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Titin-based stiffening of muscle fibers in Ehlers-Danlos Syndrome

Objective: tenascin-X (TNX) is an extracellular matrix glycoprotein whose absence leads to Ehlers-Danlos Syndrome (EDS). TNX-deficient EDS patients present with joint hypermobility and muscle weakness attributable to increased compliance of the extracellular matrix. We hypothesized that in response...

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Autores principales: Ottenheijm, Coen A. C., Voermans, Nicol C., Hudson, Bryan D., Irving, Thomas, Stienen, Ger J. M., van Engelen, Baziel G., Granzier, Henk
Formato: Artigo
Lenguaje:Inglês
Publicado: American Physiological Society 2012
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Acceso en línea:https://ncbi.nlm.nih.gov/pmc/articles/PMC3774215/
https://ncbi.nlm.nih.gov/pubmed/22223454
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1152/japplphysiol.01166.2011
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