In addition, it has been proven that some patients have autoantibodies attacking the epidermis, and desmoplakin
1 and 2 (34).
They interact with plakoglobin, desmoplakin
, and plakophilin that are involved in desmosome assembly and the anchoring of intermediate filaments of desmin .
Spheres contained extensive areas of [cTnT.sup.+] or [MyHC.sup.+] cells coexpressing adhesion molecules Desmoplakin
and Desmocollin 2, an indication of intercalated disk structures formation between cardiomyocytes (Figures 5(c), 5(d), and 5(e))).
In the Carvajal disease variety two different mutations of the desmoplakin
gene (Dsp7901del1G and DspG2375R), affecting the C-terminal of the protein, have been found as causative genes.
It has been demonstrated that during this process and in response to an extrinsic signaling mechanism that is not fully understood, these cells undergo a series of transformations, including the loss of adhesion complexes and the polarity axis (with decreased epithelial markers such as E-cadherin, desmoplakin
, and plakoglobin), hypertrophy and cytoskeleton reorganization (with the substitution of keratin filaments for vimentin).
The molecular markers foriDG include increased expression of dopamine receptor D1A (Drd1a) and decreased expression of tryptophan 2, 3-dioxygenase (Tdo2) and desmoplakin
(Dsp) in the DG of mutant mice.
Expression of desmoplakin
in the Periodontal Ligament Fibroblasts has been considered to protect gap junctions in these cells against cell transformation caused by cell contraction, which may relate to tooth movement and repair of periodontal tissues.
They are inclusive of integral proteins (desmoglein and desmocollin), cytoplasmic adapter proteins (desmoplakin
and plakoglobin) and plaque associated proteins (plakophilin, envoplakin, and periplakin).
Desmosomes consist of three major protein families- Cadherins (desmogleins and desmocollins), Armadillo (Arm) repeat proteins (plakoglobin and plakophilins) and plakin (desmoplakin
Whereas the cadherins in most AJs are linked to the actin cytoskeleton through [alpha]-and [beta]-catenin, desmosomal cadherins recruit the armadillo proteins plakoglobin and plakophilin-1, which are vertebrate-specific gene duplications related to [beta]-catenin and are linked to the intermediate filament cytoskeleton either directly or via the linker protein desmoplakin
(Schneider et al., 2003).
Additionally, the tumor is usually positive for vimentin, fascin, desmoplakin
, EGFR, and human leukocyte antigenDR.