Table 1.
Proteomic profiling of the dystrophin-deficient mdx heart.
| Proteomic study | Methods | Major findings | References |
|---|---|---|---|
| Proteomic analysis of the cardiac-specific dystrophin complex | IP-based copurification, LC-MS/MS, IB, CM | Confirmation of main dystrophin-associated proteins: dystroglycans, sarcoglycans, dystrobrevins, sarcospan, and syntrophins; plus identification of novel dystrophin-associated proteins: Cavin-1, Ahnak-1, Cypher, and Cryab | Johnson et al., 2012 [52] |
| Comparative proteomic study of 1-month to 9-month-old mdx hearts versus age-matched normal hearts | 2D-DIGE, LC-MS/MS | Differential expression of ATP synthase, serine proteinase inhibitor, glyceraldehyde-3-phosphate dehydrogenase, trifunctional enzyme, and hemoglobin | Gulston et al., 2008 [141] |
| Comparative proteomic analysis of 9-month-old mdx hearts versus age-matched normal hearts | 2D-DIGE, LC-MS/MS, IB, CM | Increased levels of lamin and nucleoside diphosphate kinase; drastic decrease in myosin light chains, tropomyosin, actin, adenylate kinase, creatine kinase, vimentin, fatty acid binding protein FABP3, isocitrate dehydrogenase, NADH dehydrogenase, myozenin, porin, and peroxiredoxin. | Lewis et al., 2010 [142] |
| Comparative proteomic analysis of 7-week-old mdx hearts versus age-matched normal hearts | Label-free MS analysis, IB | Moderate changes in young mdx hearts: actin, biglycan, troponin, protein disulphide isomerase, succinyl-CoA ligase | Holland et al., 2013 [18] |
| Proteomic analysis of the aging process in 7-week to 20-month-old mdx hearts | Label-free MS analysis, IB | Severe changes in aged mdx hearts: drastic reduction in laminin, nidogen, annexin, vimentin, ATP synthase, cytochromes, NADH dehydrogenase; increases in various IgG molecules, hydroxybutyrate dehydrogenase, ferritin, transferrin, catalase, glutathione transferase | Holland et al., 2013 [18] |
Listed are major findings from recent proteomic studies that have focused on the cardiac dystrophin-glycoprotein complex and dystrophin-deficient mdx heart tissues. Abbreviations used: 2D-DIGE: two-dimensional difference in-gel electrophoresis; CM: confocal microscopy; IB: immunoblotting; IP: immunoprecipitation; LC: liquid chromatography; MS: mass spectrometry.