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

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Item Type:	Article
Title:	Constitutive upregulations of titin-based signalling proteins in KY deficient muscles
Creators Name:	Beatham, J., Gehmlich, K., van der Ven, P.F.M., Sarparanta, J., Williams, D., Underhill, P., Geier, C., Fuerst, D.O., 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
ISSN:	0960-8966
Publisher:	Elsevier
Volume:	16
Number:	7
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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