Constitutive upregulations of titin-based signalling proteins in KY deficient muscles
| Autor: | Peter F.M. van der Ven, Christian Geier, Dieter O. Fürst, Bjarne Udd, Jane Beatham, Katja Gehmlich, Peter Underhill, Debbie Williams, Gonzalo Blanco, Jaakko Sarparanta |
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| Rok vydání: | 2006 |
| Předmět: |
Transcription
Genetic Mutant Muscle Proteins Obscurin Eccentric contractions Fight-or-flight response 03 medical and health sciences Mice 0302 clinical medicine medicine Animals Connectin Muscular dystrophy Muscle Skeletal Gene Genetics (clinical) 030304 developmental biology Oligonucleotide Array Sequence Analysis 0303 health sciences biology Nuclear Proteins LIM Domain Proteins Muscular Dystrophy Animal medicine.disease musculoskeletal system Adaptation Physiological Mice Mutant Strains Cell biology Protein Structure Tertiary Up-Regulation DNA-Binding Proteins Signalling Neurology Biochemistry Pediatrics Perinatology and Child Health biology.protein Titin Neurology (clinical) Protein Kinases 030217 neurology & neurosurgery Peptide Hydrolases Signal Transduction |
| Zdroj: | Neuromuscular disorders : NMD. 16(7) |
| ISSN: | 0960-8966 |
| Popis: | 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. |
| Databáze: | OpenAIRE |
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