Recombinant Mouse Dystrophin (Dmd), partial

1. Dp71 loss alters cerebellar synapse function and cerebellum-dependent navigation strategies without being detrimental for motor functions. PMID: 29895670
2. Influence of full-length dystrophin on brain volumes in mouse models of Duchenne muscular dystrophy PMID: 29601589
3. Impaired regenerative capacity and lower revertant fibre expansion in dystrophin-deficient Duchenne muscular dystrophy mouse muscles on DBA/2 background has been reported. PMID: 27924830
4. these results provide new insights into the spatial distribution of dystrophin glycoprotein components and their dynamics in living mice. PMID: 28364093
5. it is concluded that the LVH with high LVEDWS is associated to a degradation of dystrophin and increase of myocardial stiffness. At least in a murine model these alterations were attenuated after the administration of a matrix metalloprotease inhibitor. PMID: 28316061
6. Dystrophin-glycoprotein complex component dystroglycan 1 (Dag1) directly binds to the Hippo pathway effector Yap to inhibit cardiomyocyte proliferation in mice PMID: 28581498
7. CRIPSR-mediated genome editing efficiently excised the mutant exon 23 in dystrophic mice, restoring the expression of dystrophin protein expression in dystrophic cardiac muscles to a level approaching 40% and improving myocardial contraction. PMID: 28790199
8. We show that strong and specific expression of exogenous Dp71 in Muller cells leads to correct localization of Dp71 protein restoring all protein interactions in order to re-establish a proper functional BRB and retina homeostasis thus preventing retina from oedema. PMID: 27288449
9. avoiding vector genome loss after AAV injection by PPMO pre-treatment would allow efficient long-term restoration of dystrophin and the use of lower and thus safer vector doses for Duchenne patients. PMID: 27378686
10. To optimize a dystrophin cDNA construct for therapeutic application we designed and produced four human minidystrophins within the packaging capacity of lentiviral vectors. Two novel minidystrophins retained the centrally located neuronal nitric oxide synthase (nNOS)-anchoring domain in order to achieve sarcolemmal nNOS restoration, which is lost in most internally deleted dystrophin constructs. PMID: 27477497
11. Our study demonstrates for the first time that low-level dystrophin can partially preserve heart function. PMID: 27908661
12. Dp71 expression in hepatic cells is carried out, in part, by YY1-, Sp1- and Sp3-mediated transcription from the Dp71 promoter. PMID: 27143785
13. Deficit in cognitive flexibility was observed in mdx mice in the absence of motor dysfunction or general learning impairments. PMID: 27220066
14. Our data also support the hypothesis that altered spatial localization of GABAA receptors due to Dp427 loss is a pathological mechanism associated with brain dysfunction in DMD, suggesting that extrasynaptic GABAA receptors might be candidate targets for future therapeutic developments. PMID: 28087735
15. This finding represents the first functional evidence for a significant role of the dystrophin-associated protein complex in the regulation of Kir2.x channels. PMID: 27560040
16. Lack of dystrophin in mdx mice appears associated with defective epithelial differentiation PMID: 26868633
17. Dp71Delta78-79 dystrophin mutant stimulates neurite outgrowth in cultured neuronal cells via upregulation and phosphorylation of HspB1. PMID: 26936078
18. this study provided evidence that the Dystrophin Dp71, a membrane-associated cytoskeletal protein and the main DMD gene product in the retina, regulates astrocyte morphology and density and is associated with subsequent normal blood vessel development PMID: 26711882
19. The novel dystrophin reporter mouse provides a valuable tool for direct visualization of dystrophin expression and will allow the study of dystrophin expression in vivo and in vitro in various tissues by live cell imaging. PMID: 27382459
20. Dystrophin-based therapy improves fragility of the dystrophic skeletal muscle by preventing reduction in muscle excitability. PMID: 27441081
21. absence of dystrophin in an ApoE-null background results in attenuated atherosclerotic plaque development, accompanied by an increased proportion of differentiated SMCs and decreased proportion of T cells in plaques of an inflammatory phenotype. PMID: 26345322
22. Correlation of Utrophin Levels with the Dystrophin Protein Complex and Muscle Fibre Regeneration in Duchenne and Becker Muscular Dystrophy Muscle Biopsies. PMID: 26974331
23. We observed a significant increase in the proportion of dystrophin-positive SMFs in group of mdx mice radiation chimera 5 Gy and 3 Gy which was additionally exposed in Ca(2+)-MF in comparison with the control mdx mice PMID: 27228662
24. The full-length brain dystrophin appears to play a role during acquisition of associative learning and general processes involved in memory consolidation, but no overt involvement in working memory and/or executive functions. PMID: 26190833
25. These data define the dystrophin restoration levels required to slow down or prevent disease progression and improve overall muscle function once a dystrophic environment has been established in the mdx mouse model PMID: 25935000
26. This detailed evaluation of the SMT C1100 drug series strongly endorses the therapeutic potential of utrophin modulation as a disease modifying therapeutic strategy for all Duchenne muscular dystrophy patients irrespective of their dystrophin mutation. PMID: 25935002
27. dystrophin is also highly expressed in activated muscle stem cells (also known as satellite cells), in which it associates with the serine-threonine kinase Mark2 (also known as Par1b), an important regulator of cell polarity. PMID: 26569381
28. analysis of dual exon skipping of dystrophin and myostatin pre-mRNAs using phosphorodiamidate morpholino oligomers conjugated with an arginine-rich peptide PMID: 25959011
29. Overexpression of Galgt2 failed to inhibit skeletal muscle pathology in dystroglycan-deficient muscles, in contrast to previous studies in dystrophin-deficient mdx muscles PMID: 26435413
30. Deletion of Dp71 was associated with retinal vascular inflammation, vascular lesions with increased leukocyte adhesion and capillary degeneration. PMID: 25901007
31. Formation of dystrophin-encircled vacuoles may reflect a skeletal muscle fibre death process via a mechanism involving autophagic dysfunction in old age. PMID: 25758773
32. Reproducing Dmd exon 78 missplicing switch in mice induces muscle fibre remodelling and ultrastructural abnormalities including ringed fibres, sarcoplasmic masses or Z-band disorganization, characteristic features of myotonic dystrophy 1. PMID: 26018658
33. the 2A protease-mediated carboxyl-terminal dystrophin cleavage fragment (CtermDys) is sufficient to cause marked dystrophic cardiomyopathy. PMID: 26136477
34. DGC promotes the mechanical activation of cardiac nNOS by acting as a mechanosensor to regulate AMPK activity PMID: 26483453
35. mdx mice (lacking dystrophin) are glucose intolerant, and have increased skeletal muscle glycogen but reduced amounts of liver glycogen PMID: 24626262
36. Results showed that mdx/utrn+/- mouse develops fibrosis in both hind limb and respiratory skeletal muscles at a young age while not being so affected such that it dies prematurely, this model may be an appropriate for Duchenne muscular dystrophy. PMID: 25607927
37. Collectively, these results confirm and extend understanding of a functional role for the dystrophin-glycoprotein complex in the contractile properties of airway smooth muscle and demonstrate that this results in altered lung function in vivo. PMID: 25692961
38. Alpha-dystrobrevin is a multifunctional protein that increases dystrophin's binding to the dystrophin-glycoprotein complex. PMID: 25158611
39. results suggest that Dp71 may be involved in the functional activity of the hypothalamoneurohypophysis system PMID: 19658196
40. Defects in mitochondrial ATP synthesis in dystrophin-deficient mdx skeletal muscles may be caused by complex I insufficiency PMID: 25541951
41. dystrophin exerts an intermediary and positive role in the disuse atrophy of the slow-twitch muscles PMID: 24839113
42. Lack of Dp71 results in progressive cataract formation due to loss of fiber cell organization of the crystalline lens. PMID: 25489223
43. Data indicate that dystrophin affects caveolar integrity and airway smooth muscle contraction. PMID: 25054970
44. Dystrophin-deficient transgenic mice develop severe cardiac and respiratory dysfunction following genetic ablation of the anti-inflammatory cytokine IL-10. PMID: 24659498
45. Data presented show that dystrophin deficiency enhanced the cholinergic transmitter release evoked by nAChRs activation and caused a minor reduction of VAChT protein levels in hippocampal preparations from 12-month-old mdx mice PMID: 24704431
46. The inner limiting membrane of Dp71-null mouse is thinner and more permeable to several AAV serotypes after intravitreal injection while the Blood-Retinal Barrier of these mice remains selective for AAV particles. PMID: 24382652
47. immunocytological distribution of Dp40 was analyzed in dissociated cultured neurons, revealing that Dp40 is detected in the soma and its dendrites, but not in the axon PMID: 25152393
48. demonstrate that this isoform results from usage of an internal ribosome entry site (IRES) within exon 5 that is glucocorticoid inducible PMID: 25108525
49. Our results demonstrate that together both Dp71 and Dp260 are required for the generation of the ERG b-wave in mice. PMID: 24662427
50. Aged cryab null mice develop skeletal muscle atrophy. Cryab is a novel and necessary component of skeletal muscle RISC. PMID: 24782307

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KEGG: mmu:13405

STRING: 10090.ENSMUSP00000109633

UniGene: Mm.275608