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Constitutive upregulations of titin-based signalling proteins in KY deficient muscles

Item Type:Article
Title:Constitutive upregulations of titin-based signalling proteins in KY deficient muscles
Creators Name:Beatham, J. and Gehmlich, K. and van der Ven, P.F.M. and Sarparanta, J. and Williams, D. and Underhill, P. and Geier, C. and Fuerst, D.O. and Udd, B. and Blanco, G.
Abstract:An increase in the expression of stretch/stress response elements in fast and slow muscles has been previously described in a transcriptional profiling of KY deficient muscles. Here, we have characterized the induction of this titin-based family of signalling proteins in ky/ky muscles at the protein level. Changes in expression of MLP, MARP2 and Xin have been related to the onset of dystrophic and adaptive changes that operate in ky/ky muscles. Our results indicate that induction of this set of genes is an early consequence of the interference caused by the absence of the KY protein. A search of muscle profiles of mouse models revealed such molecular hallmark only in muscles subjected to a single bout of eccentric contractions and specific titin mutants. Based on the role of this family as titin-based stress response molecules, it is suggested that titin structural/signalling instability is common to ky and titin mouse mutants and eccentric contractions.
Keywords:Muscular Dystrophy, Transcriptional Profiling, Titinopathies, Animals, Mice
Source:Neuromuscular Disorders
Page Range:437-445
Date:July 2006
Official Publication:https://doi.org/10.1016/j.nmd.2006.05.013
PubMed:View item in PubMed

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