{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,5]],"date-time":"2026-10-05T20:33:29Z","timestamp":1791232409747,"version":"4.1.0"},"reference-count":55,"publisher":"Wiley","issue":"16","license":[{"start":{"date-parts":[[2006,7,11]],"date-time":"2006-07-11T00:00:00Z","timestamp":1152576000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["analyticalsciencejournals.onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Proteomics"],"published-print":{"date-parts":[[2006,8]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Duchenne muscular dystrophy is the most commonly inherited neuromuscular disorder in humans. Although the primary genetic deficiency of dystrophin in X\u2010linked muscular dystrophy is established, it is not well\u2010known how pathophysiological events trigger the actual fibre degeneration. We have therefore performed a DIGE analysis of normal diaphragm muscle <jats:italic>versus<\/jats:italic> the severely affected x\u2010linked muscular dystrophy (MDX) diaphragm, which represents an established animal model of dystrophinopathy. Out of 2398\u2005detectable 2\u2010D protein spots, 35\u2005proteins showed a drastic differential expression pattern, with 21\u2005proteins being decreased, including Fbxo11\u2010protein, adenylate kinase, \u03b2\u2010haemoglobin and dihydrolipoamide dehydrogenase, and 14\u2005proteins being increased, including cvHSP, aldehyde reductase, desmin, vimentin, chaperonin, cardiac and muscle myosin heavy chain. This suggests that lack of sarcolemmal integrity triggers a generally perturbed protein expression pattern in dystrophin\u2010deficient fibres. However, the most significant finding was the dramatic increase in the small heat shock protein cvHSP, which was confirmed by 2\u2010D immunoblotting. Confocal fluorescence microscopy revealed elevated levels of cvHSP in MDX fibres. An immunoblotting survey of other key heat shock proteins showed a differential expression pattern in MDX diaphragm. Stress response appears to be an important cellular mechanism in dystrophic muscle and may be exploitable as a new approach to counteract muscle degeneration.<\/jats:p>","DOI":"10.1002\/pmic.200600082","type":"journal-article","created":{"date-parts":[[2006,7,11]],"date-time":"2006-07-11T08:52:29Z","timestamp":1152607949000},"page":"4610-4621","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":105,"title":["Proteome analysis of the dystrophin\u2010deficient MDX diaphragm reveals a drastic increase in the heat shock protein cvHSP"],"prefix":"10.1002","volume":"6","author":[{"given":"Philip","family":"Doran","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Geraldine","family":"Martin","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Paul","family":"Dowling","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Harald","family":"Jockusch","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kay","family":"Ohlendieck","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2006,8,8]]},"reference":[{"key":"e_1_2_1_2_2","volume-title":"Methods in Molecular Biology","author":"Cutler P.","year":"2004"},{"key":"e_1_2_1_3_2","volume-title":"Myology: Basic and Applied","author":"Engel A. 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