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DES encodes a muscle-specific class III intermediate filament. Additionally we are shipping Desmin Antibodies (413) and Desmin Proteins (33) and many more products for this protein.
Showing 10 out of 73 products:
Human Desmin ELISA Kit for Sandwich ELISA - ABIN417384
Kobayashi, Olson, Sly, Swanson, Chung, Naryshkin, Narasimhan, Bhattacharyya, Mullenix, Chen: Utility of survival motor neuron ELISA for spinal muscular atrophy clinical and preclinical analyses. in PLoS ONE 2011
Show all 3 Pubmed References
Human Desmin ELISA Kit for Sandwich ELISA - ABIN1379903
Tidball: Desmin at myotendinous junctions. in Experimental cell research 1992
Desmin intermediate filaments are required for normal active force generation.
Desmin distribution during muscle maturation changes from dispersed aggregates to a perinuclear concentration to striated (show NSDHL ELISA Kits) afterwards.
A novel mutation (c.679 C>T /p.R227C) in exon 3 of DES was identified and cosegregated with the affected members of a Chinese family with isolated Dilated cardiomyopathy (DCM) phenotypes (left ventricle and left atrial diameters).
Desmin, Glial Fibrillary Acidic Protein (show GFAP ELISA Kits), Vimentin (show VIM ELISA Kits), and Peripherin (show PRPH ELISA Kits) are type III intermediate filaments that have roles in health and disease [review]
Phenotypic expression of a novel desmin gene mutation: hypertrophic cardiomyopathy followed by systemic myopathy.
Using a combination of co-sedimentation centrifugation, viscometric assays and electron microscopy of negatively stained filaments to analyse the in vitro assembly of desmin filaments, this study shows that the binding of CRYAB (show CRYAB ELISA Kits) to desmin is subject to its assembly status, to the subunit organization within filaments formed and to the integrity of the C-terminal tail domain of desmin.
Targeted sequencing revealed trigenic mutations: c.700G>A/p.E234K in DES, c.2966G>A/p.R989H in MYPN (show MYPN ELISA Kits), and c.5918G>C/p.R1973P in CACNA1C (show CACNA1C ELISA Kits) in a family of hypertrophic cardiomyopathy with early repolarization and short QT syndrome.
demonstrate that the expression of mutant desmin causes disruption of the extrasarcomeric desmin cytoskeleton and extensive mitochondrial abnormalities regarding subcellular distribution, number and shape
Mutation in the Core Structure of Desmin Intermediate Filaments Affects Myoblast Elasticity
Data show that the filament elongation of both desmin and keratin K8/K18 (show KRT18 ELISA Kits) proceeds very similar to that of vimentin (show VIM ELISA Kits).
Cdk1 (show CDK1 ELISA Kits)-induced desmin phosphorylation is required for efficient separation of desmin-IFs and generally detected in muscular mitotic cells in vivo.
Desmin, Lamin A/C, MMP9, and histone H4 were upregulated in the placental villi of women experiencing early pregnancy loss.
Data suggest that mutated desmin markedly impedes myocyte structure and function at pre-symptomatic stages of myofibrillar myopathies.
Moderate MnSOD (show SOD2 ELISA Kits) and/or catalase (show CAT ELISA Kits) overexpression in desmin-null hearts leads to a marked decrease in intracellular reactive oxygen species, ameliorates mitochondrial and other ultrastructural defects, minimizes myocardial degeneration and leads to a significant improvement of cardiac function.
R349P mutant desmin-related remodeling of the 3D myofibrillar architecture during aging, which provides a structural basis for the progressive muscle weakness.
The disease-mutant desmin variants E245D and T453I exhibited increased binding affinity for nebulette (show NEBL ELISA Kits), delayed filament assembly kinetics, and caused significant weakening of networks.
desmin and alphaBeta-crystallin are localized at sarcoplasmic reticulum (SR)-mitochondria-associated membranes (MAMs), where they interact with VDAC, Mic60 - the core component of mitochondrial contact site and cristae organizing system (MICOS) complex - and ATP synthase, suggesting that these associations could be crucial in mitoprotection at different levels.
Complete lack or markedly decreased expression of mutant R349P desmin impairs the structural and functional integrity of neuromuscular endplates.
the coordinated up-regulations of desmin and alpha- actinin (show ACTN1 ELISA Kits) specifically in the early stage of diastolic heart failure mouse models indicate a novel myocardial response.
E413K mutation induces desmin network disorganization, desmin aggregate formation and alters the traction forces generation of single myoblasts.
Results show that expression of desmin increased during the late fattening stage of of hanwoo steers and it contributes to the muscle contractile apparatus.
This gene encodes a muscle-specific class III intermediate filament. Homopolymers of this protein form a stable intracytoplasmic filamentous network connecting myofibrils to each other and to the plasma membrane. Mutations in this gene are associated with desmin-related myopathy, a familial cardiac and skeletal myopathy (CSM), and with distal myopathies.
, desmin, gene 1
, intermediate filament protein
, muscle-specific intermediate filament desmin