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Muscle degeneration in the heart of 1-9 month-old mdx mice (a model for Duchenne muscular dystrophy) has been monitored using metabolomic and proteomic approaches. In both data sets, a pronounced aging trend was detected in control and mdx mice, and this trend was separate from the disease process. ...
2017-03-14 | MTBLS256 | MetaboLights
Goal was to assess protein linkers between microtubules and dystrophin (specifically dystrophin regions R4-15 and R20-23). Two paired 10-plex TMT experiments were used to compare sixteen unique Dystrophin Glycoprotein Complex (or microtubule) enrichments (four genotypes, each with n=4) plus four poo...
ORGANISM(S): Mus musculus (Mouse) 
2020-09-21 | PXD021088 | Pride
Crossing of hDMD mice that contain the full-length 2.3 Mb hDMD gene were crossed with dystrophin-deficient mdx mice and dystrophin and utrophin double-deficient mdx x utrn-/- mice resulted in a full rescue of the dystrophic features of these mice, as concluded from histological analysis. Analysis on...
ORGANISM(S): Mus musculus 
Dystrophin was knocked down in primary muscle cultures prepared from C57Bl/10 neonate mice using siRNA targeting dystrophin. Also in parallel, primary muscle cultures treated with siRNA targting luciferase were used as controls. The experiment was designed so that the same cell populations were used...
ORGANISM(S): Mus musculus 
Duchenne Muscular Dystrophy (DMD) is a fatal muscle wasting disorder caused by dystrophin deficiency. Previous work suggested that increased expression of the dystrophin-related protein utrophin in the mdx mouse model of DMD can prevent dystrophic pathophysiology. Physiological tests showed that t...
ORGANISM(S): Mus musculus 
Mapping of dystrophin-lipid interactions by mass spectrometry
ORGANISM(S): Homo sapiens (Human) 
2019-11-14 | PXD007716 | Pride
Comparison by expression profiling of tissue from dKO (utrophin/dystrophin-deficient) and MDX mice at 8 weeks of age. Independent triplicate analyses/strain were done for extraocular, hindlimb, and cardiac muscle.
ORGANISM(S): Mus musculus 
Dystrophin proteomic regulation in Muscular Dystrophies (MD) remains unclear. We report that a long noncoding RNA (lncRNA) H19 associates with dystrophin. To investigate the biological roles of this interaction in vivo, we performed mass spectrometry analysis of dystrophin and its associated protein...
ORGANISM(S): Mus musculus (Mouse) 
2020-08-19 | PXD020566 | Pride
Duchenne muscular dystrophy (DMD) is a severely debilitating and incurable neuromuscular disease. Its conspicuous feature is the absence of dystrophin in myofibers and therefore most therapeutic approaches focus on some form of its re-expression there. However, increasing body of evidence points at ...
ORGANISM(S): Mus musculus 
Determination of gene expression changes in extraocular and hindlimb (gastrocnemius/soleus) of mdx (dystrophin-deficient) mice at postnatal day 56. 5 independent replicates/muscle group/strain.
ORGANISM(S): Mus musculus 
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