دورية أكاديمية
Proteome analysis of the dystrophin-deficient MDX diaphragm reveals a drastic increase in the heat shock protein cvHSP
| العنوان: | Proteome analysis of the dystrophin-deficient MDX diaphragm reveals a drastic increase in the heat shock protein cvHSP |
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| المؤلفون: | Doran, Philip, Martin, Geraldine, Dowling, Paul, Jockusch, Harald, Ohlendieck, Kay |
| بيانات النشر: | WILEY-V C H VERLAG GMBH |
| سنة النشر: | 2006 |
| المجموعة: | PUB - Publications at Bielefeld University |
| مصطلحات موضوعية: | dystrophy, skeletal muscle proteomics, DIGE, cardiovascular heat shock protein, muscular, MDX diaphragm |
| الوصف: | Doran P, Martin G, Dowling P, Jockusch H, Ohlendieck K. Proteome analysis of the dystrophin-deficient MDX diaphragm reveals a drastic increase in the heat shock protein cvHSP. PROTEOMICS . 2006;6(16):4610-4621. ; Duchenne muscular dystrophy is the most commonly inherited neuromuscular disorder in humans. Although the primary genetic deficiency of dystrophin in X-linked muscular dystrophy is established, it is not well-known how pathophysiological events trigger the actual fibre degeneration. We have therefore performed a DIGE analysis of normal diaphragm muscle versus the severely affected x-linked muscular dystrophy (MDX) diaphragm, which represents an established animal model of dystrophinopathy. Out of 2398 detectable 2-D protein spots, 35 proteins showed a drastic differential expression pattern, with 21 proteins being decreased, including Fbxol 1-protein, adenylate kinase, beta-haemoglobin and dihydrolipoarnide dehydrogenase, and 14 proteins 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-deficient fibres. However, the most significant finding was the dramatic increase in the small heat shock protein cvHSP, which was confirmed by 2-D 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. |
| نوع الوثيقة: | article in journal/newspaper |
| اللغة: | English |
| العلاقة: | info:eu-repo/semantics/altIdentifier/issn/1615-9853; info:eu-repo/semantics/altIdentifier/issn/1615-9861; info:eu-repo/semantics/altIdentifier/wos/000240036100016; info:eu-repo/semantics/altIdentifier/pmid/16835851; https://pub.uni-bielefeld.de/record/1598094Test |
| الإتاحة: | https://doi.org/10.1002/pmic.200600082Test https://pub.uni-bielefeld.de/record/1598094Test |
| حقوق: | info:eu-repo/semantics/closedAccess |
| رقم الانضمام: | edsbas.186397F6 |
| قاعدة البيانات: | BASE |
| الوصف غير متاح. |