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PANoptosis is a prominent feature of desmoplakin cardiomyopathy
Introduction: Arrhythmogenic cardiomyopathy (ACM) is hereditary cardiomyopathy caused by pathogenic variants (mutations) in genes encoding the intercalated disc (ID), particularly desmosome proteins. ACM caused by mutations in the DSP gene encoding desmoplakin (DSP) is characterized by the prominence of cell death, myocardial fibrosis, and inflammation,
Hyun-Hwan Jeong +2 more
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Identification and characterization of DSPIa, a novel isoform of human desmoplakin [PDF]
Desmoplakin is a ubiquitous component of desmosomes and desmosome-like structures, such as the cardiomyocyte area composita. Two major isoforms, desmoplakin I (DSPI) and desmoplakin II (DSPII) are encoded by alternative mRNA transcripts differentially ...
David Kelsell, Rita M Cabral, Hong Wan
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Intracellular Distribution of Desmoplakin in Human Odontoblasts [PDF]
Coexpression of desmosomal proteins and vimentin has been reported in a specific mesenchymal phenotype. This study investigated the expression of vimentin-binding desmosomal proteins in human dental pulp fibroblasts (DPF) and odontoblasts. The dental pulp has no cells expressing desmocollin (DSC) 1–3, desmoglein (DSG) 1–3, junction plakoglobin (JUP ...
Yoshihiko Sawa +2 more
exaly +3 more sources
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Desmoplakin as a Specific Marker of Lymphatic Vessels
Microvascular Research, 2001The usefulness of immunostaining with anti-desmoplakin antibody for light microscopic identification of lymphatic vessels was examined in cryostat sections of the human tongue. The results were compared with laminin, 5'-nucleotidase (5'-Nase), and factor VIII staining.
Yoshihiko Sawa +2 more
exaly +3 more sources
Desmoplakin Variants Are Associated with Idiopathic Pulmonary Fibrosis [PDF]
Abstract Rationale Sequence variation, methylation differences, and transcriptional changes in desmoplakin (DSP) have been observed in patients with idiopathic pulmonary fibrosis (IPF). Objectives To identify novel ...
David A Schwartz +2 more
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Purification and Structural Analysis of Desmoplakin
2016Desmoplakin (DP) is an obligate component of desmosomes, where it links the desmosomal cadherin/plakoglobin/plakophilin assembly to intermediate filaments. DP contains a large amino-terminal domain (DPNT) that binds to the cadherin/plakoglobin/plakophilin complex, a central coiled-coil domain that dimerizes the molecule, and a C-terminal domain (DPCT ...
Hee-Jung, Choi, William I, Weis
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Antibodies to desmoplakin in a patient with pemphigus foliaceous
Journal of the European Academy of Dermatology and Venereology, 1998AbstractBackgroundAntibodies to desmoplakins have been associated with paraneoplastic pemphigus. Here, we report a case of non‐neoplastic pemphigus foliaceous with antibodies to desmoplakins.ObservationA 61‐year‐old woman with pemphigus foliaceous by clinical, histological and immunofiuorescent criteria was found to have circulating antibodies to 250 ...
D, Jiao, J C, Bystryn
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Desmoplakin is essential in epidermal sheet formation
Nature Cell Biology, 2001We have generated an epidermis-specific desmoplakin (DP) mouse knockout, and show that epidermal integrity requires DP; mechanical stresses to DP-null skin cause intercellular separations. The number of epidermal desmosomes in DP-null skin is similar to wild type (WT), but they lack keratin filaments, which compromise their function.
V, Vasioukhin +4 more
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Desmoplakin II expression is not restricted to stratified epithelia
Journal of Cell Science, 1990ABSTRACT Desmosomes are major intercellular junctions found in association with intermediate filaments in epithelial, cardiac and arachnoidal tissue. Desmoplakins I and II (DPI and II) are highly related proteins localized in the innermost part of the desmosomal plaque and are candidates for linking intermediate filaments (IF) to the ...
B D, Angst, L A, Nilles, K J, Green
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