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

Journal article

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.

Authors: Beatham, J; Gehmlich, K; van der Ven, P; Sarparanta, J; Williams, D

• Journal: Neuromuscular disorders : NMD
• Volume: 16
• Issue: 7
• Pages: 437-445
• Publication date: 2006-07-01
• DOI: 10.1016/j.nmd.2006.05.013
• EISSN: 1873-2364
• ISSN: 0960-8966
• Language: English
• Keywords: Nuclear Proteins; Muscle, Skeletal; Animals; Adaptation, Physiological; Signal Transduction; Connectin; Muscle Proteins; LIM Domain Proteins; Mice; Oligonucleotide Array Sequence Analysis; Protein Kinases; DNA-Binding Proteins; Transcription, Genetic; Mice, Mutant Strains; Muscular Dystrophy, Animal; Up-Regulation; Protein Structure, Tertiary