| First Author | Janke A | Year | 2013 |
| Journal | Dev Dyn | Volume | 242 |
| Issue | 12 | Pages | 1369-81 |
| PubMed ID | 23940011 | Mgi Jnum | J:203003 |
| Mgi Id | MGI:5523750 | Doi | 10.1002/dvdy.24031 |
| Citation | Janke A, et al. (2013) A new look at cytoskeletal NOS-1 and beta-dystroglycan changes in developing muscle and brain in control and mdx dystrophic mice. Dev Dyn 242(12):1369-81 |
| abstractText | Background: Loss of dystrophin profoundly affects muscle function and cognition. Changes in the dystrophin-glycoprotein complex (DGC) including disruption of nitric oxide synthase (NOS-1) may result from loss of dystrophin or secondarily after muscle damage. Disruptions in NOS-1 and beta-dystroglycan (bDG) were examined in developing diaphragm, quadriceps, and two brain regions between control and mdx mice at embryonic day E18 and postnatal days P1, P10, and P28. Age-dependent differential muscle loading allowed us to test the hypothesis that DGC changes are dependent on muscle use. Results: Muscle development, including loss of central nucleation and the localization of NOS-1 and bDG, was earlier in diaphragm than quadriceps; these features were differentially disrupted in dystrophic muscles. The NOS-1/bDG ratio, an index of DGC stability, was higher in dystrophic diaphragm (P10-P28) and quadriceps (P28) than controls. There were also distinct regional differences in NOS-1 and bDG in brain tissues with age and strain. NOS-1 increased with age in control forebrain and cerebellum, and in mdx cerebellum; NOS-1 and bDG were higher in control than mdx mouse forebrain. Conclusions: Important developmental changes in structure and muscle DGC preceded the hallmarks of dystrophy, and are consistent with the impact of muscle-specific differential loading during maturation. Developmental Dynamics 242:1369-1381, 2013. (c) 2013 Wiley Periodicals, Inc. |
