Desmoplakin and clinical manifestations of desmoplakin cardiomyopathy
. Desmoplakin (DSP), encoded by the DSP gene, is the main desmosome component and is abundant in the myocardial tissue. There are three DSP isoforms that assume the role of supporting structural stability through intercellular adhesion. It has been found
Zhong-Yu Yuan +4 more
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Paraneoplastic pemphigus (PNP) is an autoimmune bullous disease associated with underlying neoplasms and characterized by antibodies against desmoglein 3 (Dsg 3) and plakins.
Xue Wang +28 more
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Desmoplakin cardiomyopathy: case report. [PDF]
Helal A, Alama M, Ali W, Farooq M.
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Humanized Dsp ACM Mouse Model Displays Stress-Induced Cardiac Electrical and Structural Phenotypes
Arrhythmogenic cardiomyopathy (ACM) is an inherited disorder characterized by fibro-fatty infiltration with an increased propensity for ventricular arrhythmias and sudden death. Genetic variants in desmosomal genes are associated with ACM.
Tyler L. Stevens +14 more
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BackgroundPathogenic/Likely pathogenic variants in DSP-encoded desmoplakin are strongly associated with arrhythmogenic cardiomyopathy (ACM). However, their contribution towards sinus node dysfunction has not been well-delineated.Case summaryA 74-year-old
Nicholas Y. Tan +4 more
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Desmoplakin interacts with the coil 1 of different types of intermediate filament proteins and displays high affinity for assembled intermediate filaments. [PDF]
The interaction of intermediate filaments (IFs) with the cell-cell adhesion complexes desmosomes is crucial for cytoskeletal organization and cell resilience in the epidermis and heart.
Bertrand Favre +6 more
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Desmosomal Junctions and Connexin-43 Remodeling in High-Pacing-Induced Heart Failure Dogs
Background: While desmosomal junctions and gap junction remodeling are among the arrhythmogenic substrates, the fate of desmosomal and gap junctions in high-pacing-induced heart failure remains unclear. This aim of this study was to determine the fate of
Qing Wang +6 more
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Cortical tension regulates desmosomal morphogenesis
Mechanical stability is a fundamental and essential property of epithelial cell sheets. It is in large part determined by cell-cell adhesion sites that are tightly integrated by the cortical cytoskeleton.
Marcin Moch +2 more
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FDG PET/CT imaging and circulating biomarkers of inflammation in desmoplakin cardiomyopathy. [PDF]
Abstract Aims Inflammation has been implicated in the pathogenesis of desmoplakin (DSP) cardiomyopathy, and retrospective studies have described abnormal myocardial fluorodeoxyglucose (FDG) positron emission tomography/computed tomography (PET/CT) findings in symptomatic patients eventually diagnosed with DSP cardiomyopathy.
Divakaran S +10 more
europepmc +2 more sources
Genetic deletion of the desmosomal component desmoplakin promotes tumor microinvasion in a mouse model of pancreatic neuroendocrine carcinogenesis. [PDF]
We used the RIP1-Tag2 (RT2) mouse model of islet cell carcinogenesis to profile the transcriptome of pancreatic neuroendocrine tumors (PNET) that were either non-invasive or highly invasive, seeking to identify pro- and anti-invasive molecules ...
Matthew G H Chun, Douglas Hanahan
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