Recombinant Mouse Desmin (Des)

1. Data suggest that mutated desmin markedly impedes myocyte structure and function at pre-symptomatic stages of myofibrillar myopathies. PMID: 28469177
2. R349P mutant desmin-related remodeling of the 3D myofibrillar architecture during aging, which provides a structural basis for the progressive muscle weakness. PMID: 28715662
3. demonstrate that the expression of mutant desmin causes disruption of the extrasarcomeric desmin cytoskeleton and extensive mitochondrial abnormalities regarding subcellular distribution, number and shape PMID: 27393313
4. The disease-mutant desmin variants E245D and T453I exhibited increased binding affinity for nebulette, delayed filament assembly kinetics, and caused significant weakening of networks. PMID: 27733623
5. Complete lack or markedly decreased expression of mutant R349P desmin impairs the structural and functional integrity of neuromuscular endplates. PMID: 27440146
6. the coordinated up-regulations of desmin and alpha- actinin specifically in the early stage of diastolic heart failure mouse models indicate a novel myocardial response. PMID: 26529187
7. E413K mutation induces desmin network disorganization, desmin aggregate formation and alters the traction forces generation of single myoblasts. PMID: 26789769
8. This study identifies desmin as a new Asb2b target for proteasomal degradation in cardiomyocytes and suggests that accumulation of desmin could contribute to UPS impairment in Hypertrophic cardiomyopathy mice and patients PMID: 26343497
9. found that inhibition of the Rac1 pathway (a G protein signaling pathway involved in diverse cellular processes), antioxidant treatment, and stimulation of macroautophagy reduced desmin aggregation by up to 75% in this model PMID: 26333167
10. This demonstration of biomechanical integration by the desmin intermediate filament system suggests that it plays an active biological role in muscle in addition to its accepted structural role PMID: 25413344
11. expression level of mutant versus wild-type desmin in mouse model as well as in skeletal muscle specimens derived from human R350P desminopathies; findings demonstrate missense-mutant desmin inflicts changes of the subcellular localization and turnover of desmin itself and of direct desmin-binding partners PMID: 25394388
12. Disruption of both nesprin 1 and desmin results in decreased lifespan, body weight and muscle strength. PMID: 24943590
13. The desmin intermediate filament network plays a major role in striated muscle development and maintenance by integrating and coordinating most cellular components necessary for proper mechanochemical signaling, organelle cross-talk, energy production and trafficking processes required for proper tissue homeostasis. [Review] PMID: 25680090
14. Desmin content and transversal stiffness of the left ventricle mouse cardiomyocytes and skeletal muscle fibers after a 30-day space flight on board "BION-M1" biosatellite PMID: 25730983
15. the observed decline in [Ca(2+)]mit was due to desmin aggregate accumulation resulting in the loss of desmin mitochondria interactions PMID: 25171807
16. expression detected specifically in the mylohyoid, strong expression evident after embryonic day 13, increased with age PMID: 22476899
17. During fasting, desmin phosphorylation increases and enhances Trim32-mediated degradation of the desmin cytoskeleton, which appears to facilitate the breakdown of Z-bands and thin filaments. PMID: 22908310
18. The number of malformed myofibers in mice lacking desmin, keratin 19, or both intermediate filament proteins (double knockout, DKO) is increased and coincident with altered excitation-contraction coupling calcium ion (Ca2+) kinetics. PMID: 22592402
19. These data suggest a potential link between increased cellular damage and the development of fibrosis in muscles lacking the cytoskeletal support of the desmin filament network. PMID: 22442138
20. disarrangement of desmin, at least partly related to inadequate phosphorylation of alphaB-crystallin seems to be importantly involved in the progressive deterioration of contractile heart function. PMID: 22285482
21. the structural response of each genotype is distinct, suggesting multiple mechanisms by which desmin and keratin influence the biomechanical properties of myofibers PMID: 22287836
22. Desmin regulates airway smooth muscle hypertrophy through early growth-responsive protein-1 and microRNA-26a. PMID: 21903578
23. role in nebulin Z-disc assembly and actin thin filament organization PMID: 21984811
24. we found significantly increased autophagic flux in mouse hearts with transgenic overexpression of a cardiomyopathy-linked mutant desmin PMID: 21659648
25. the state of desmin-filament assembly is crucial for synemin anchorage and consequently might involve mechanical and functional stability of the cytoskeletal network PMID: 21262226
26. Results suggest that keratin filaments containing K19- and desmin-based intermediate filaments can play independent, complementary, or antagonistic roles in the physiology and morphology of fast-twitch skeletal muscle. PMID: 21209367
27. mutations in MTM1 disrupted the MTM1-desmin complex, resulting in abnormal intermediate filament assembly and architecture in muscle cells PMID: 21135508
28. Effects of desmin gene knockout on mice heart mitochondria. PMID: 11710808
29. In myocarditis, expression of connexin 43 and desmin in the involved cardiac muscle cells was inhibited, resulting in dysfunction of gap junctional communication and arrhythmia. PMID: 11866927
30. extensive fibrosis and dystrophic calcification observed in the heart of desmin-null mice, potentially crucial events leading to heart failure PMID: 11891192
31. Mechanical function of intermediate filaments in arteries of different size examined using knockout mice PMID: 11986381
32. Results suggest that desmin associates with and selectively stabilizes the Z line domains of costameres, but that cytokeratins associate with all three domains of costameres, even in the absence of desmin. PMID: 12134074
33. Mice lacking the desmin gene display excessive microvascular adaptation to changes in blood flow through the mesenteric arteries: flow-dependent dilation is higher in high-flow arteries and lower in low-flow arteries than in control arteries. PMID: 12377733
34. N-desmin has a role as a dominant-negative inhibitor of filament assembly, both for desmin and the structurally related intermediate filament protein vimentin PMID: 12477713
35. Lack of desmin adversely affects ability of mice to engage in both chronic and acute bouts of endurance running exercise, but this decrement in performance is not associated with increase in serum creatine kinase activity. PMID: 12844497
36. desmin has roles in mouse skeletal muscle during passive deformation PMID: 15111414
37. Data show that different desmin mutants interfere with intermediate filament assembly at distinct stages. PMID: 16217025
38. Desmin modulates lung compliance, lung recoil pressure, and airway contractile response. PMID: 16387753
39. Results suggest that the positioning of nuclei along muscle fibers is plastic and that blood vessels and desmin intermediate filaments each play a distinct role in the control of this positioning. PMID: 16972267
40. Constitutive expression of desmin and increased synthesis during mesoderm formation leads to up-regulation of brachyury and nkx2.5 genes, accelerated early cardiomyogenesis, and results in the development of cardiomyocyte clusters. PMID: 17381547
41. Des was detected immunohistochemically in wild type mice, but not knockout mouse. PMID: 17612291
42. perinuclear localization of the TRIM-like protein myospryn requires its binding partner desmin PMID: 17872945
43. Fetal muscle cells express a higher density of muscle markers, such as desmin, than that of young and adult cells. PMID: 17962961
44. ability of Surfactant protein A to interact with desmin and vimentin, and to prevent polymerization of desmin monomers, shed light on unexpected and wider biological roles of this collectin PMID: 18407667
45. These findings establish plectin as the major organizer of desmin IFs in myofibers and provide new insights into plectin- and desmin-related muscular dystrophies. PMID: 18490514
46. Desmin mediates TNF-alpha-induced aggregate formation and intercalated disk reorganization in heart failure PMID: 18519735
47. Analysis of hearts from transgenic animals revealed that mutant desmin loses its Z-disc localization but can still associate with the intercalated discs, which, however, have an altered architecture, resembling other examples of dilated cardiomyopathy. PMID: 18539904
48. Desmin in the bladder smooth muscle is not needed for adaptive growth (cell enlargement) but has a role in active force transmission and maintenance of wall structure. PMID: 18562479
49. results imply that low levels of L345P desmin mutation acts, at least partially, by a dominant negative effect on mitochondria PMID: 18563598
50. DesQ389P and DesD399Y may cause muscle disease by altering the specific biophysical properties of the desmin filaments, thereby compromising both its mechanosensing and mechanotransduction ability. PMID: 19026658

Show More

Hide All

KEGG: mmu:13346

STRING: 10090.ENSMUSP00000027409

UniGene: Mm.6712