Results 11 to 20 of about 11,754 (266)
Desmoplakin and clinical manifestations of desmoplakin cardiomyopathy
Chinese Medical Journal, 2021 . 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
doaj +6 more sources
Desmoplakin and features of desmoplakin cardiomyopathy
Российский кардиологический журнал, 2023 Inherited cardiomyopathies (CMP) are a group of heterogeneous diseases characterized by myocardial disorders that is not caused by coronary artery disease, hypertension, valvular and congenital defects.
R. P. Myasnikov +11 more
doaj +3 more sources
Desmoplakin CSM models unravel mechanisms regulating the binding to intermediate filaments and putative therapeutics for cardiocutaneous diseases [PDF]
Scientific ReportsArrhythmogenic cardiomyopathy (AC) is a common cause of sudden cardiac arrest and death in young adults. It can be induced by different types of mutations throughout the desmoplakin gene including the R2834H mutation in the extreme carboxyterminus tail ...
Cedric Badowski +3 more
doaj +3 more sources
Frontiers in NeuroscienceDesmoplakin (Dsp) is a component of desmosomal cell–cell junctions that interacts with the cadherin complex and cytoskeletal intermediate filaments. In addition to its function as an adhesion component, Dsp is involved in various biological processes ...
Keisuke Otsubo +7 more
doaj +3 more sources
Structure of the Intermediate Filament-Binding Region of Desmoplakin. [PDF]
PLoS ONE, 2016 Desmoplakin (DP) is a cytoskeletal linker protein that connects the desmosomal cadherin/plakoglobin/plakophilin complex to intermediate filaments (IFs).
Hyunook Kang +4 more
doaj +6 more sources
Desmoplakin and periplakin genetically and functionally contribute to eosinophilic esophagitis [PDF]
Nature Communications, 2021 Eosinophilic esophagitis (EoE) is a chronic allergic inflammatory disease with a complex underlying genetic etiology. Here, the authors identify a series of rare variants in DSP and PPL in multiplex families with EoE and uncover a pathogenic role for ...
Tetsuo Shoda +20 more
doaj +4 more sources
Clinical characteristics and risk stratification of desmoplakin cardiomyopathy [PDF]
EP Europace, 2021 Aims Desmoplakin (DSP) cardiomyopathy is an increasingly recognized form of arrhythmogenic cardiomyopathy. With a genotype-specific approach, we characterized the diagnosis, natural history, and risk for ventricular arrhythmia and heart failure in DSP ...
Weijia Wang +9 more
semanticscholar +4 more sources
PANoptosis is a prominent feature of desmoplakin cardiomyopathy
The Journal of Cardiovascular Aging, 2022 Introduction: Arrhythmogenic cardiomyopathy (ACM) is hereditary cardiomyopathy caused by pathogenic variants (mutations) in genes encoding the intercalated disc (ID), particularly desmosome proteins.
Melis Olcum +7 more
semanticscholar +3 more sources
Arrhythmogenic Cardiomyopathy—Further Insight into the Clinical Spectrum of Desmoplakin Disease [PDF]
Cardiogenetics, 2021 Arrhythmogenic cardiomyopathy is a familial heart muscle disease characterized by structural, electrical, and pathological abnormalities. Recognition of left ventricular (LV) involvement in arrhythmogenic right ventricular cardiomyopathy (ARVC) has led ...
Joanne Simpson +5 more
doaj +2 more sources
Frontiers in Immunology, 2022 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
doaj +2 more sources