Proteomic Profiling of the Dystrophin-Deficient mdx Phenocopy of Dystrophinopathy-Associated Cardiomyopathy

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.