Desmoplakin

Cell adhesion in desmosomes

Protein in humans that is encoded by the DSP gene.

- Desmoplakin

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Desmosome

Cell structure specialized for cell-to-cell adhesion.

A desmosome.

The outer dense plaque, which is about 15–20 nm in length, contains the intracellular ends of desmocollin and desmoglein, the N-terminus side of desmoplakin, and the armadillo family of mediatory proteins plakoglobin and plakophilin.

Palmoplantar keratoderma

Palmoplantar keratodermas are a heterogeneous group of disorders characterized by abnormal thickening of the stratum corneum of the palms and soles.

Patient with severe plantar keratosis.
Diffuse palmoplantar keratoderma

Type 2: : DSP

Vimentin

Structural protein that in humans is encoded by the VIM gene.

Immunofluorescence staining pattern of vimentin antibodies. Produced by incubating vimentin primary antibodies and FITC labelled secondary antibodies with HEp-20-10 cells.
Immunofluorescence staining of HeLa Cells with antibody to reveal vimentin containing intermediate filaments in green and antibody to LAMP1 to reveal lysosomes in red. Nuclear DNA is seen in blue. Antibodies and image courtesy EnCor Biotechnology Inc.

DSP

Desmin

Protein that in humans is encoded by the DES gene.

A representation of the 3D structure of the protein myoglobin showing turquoise α-helices. This protein was the first to have its structure solved by X-ray crystallography. Toward the right-center among the coils, a prosthetic group called a heme group (shown in gray) with a bound oxygen molecule (red).

Desmin has been shown to interact with desmoplakin and αB-crystallin.

Intermediate filament

Intermediate filaments (IFs) are cytoskeletal structural components found in the cells of vertebrates, and many invertebrates.

Structure of lamin a/c globular domain
Structure of intermediate filament
Human vimentin coil 2b fragment (cys2)
Keratin intermediate filaments (stained red) around epithelial cells
Vimentin fibers in fibroblasts

Keratin filaments in epithelial cells link to desmosomes (desmosomes connect the cytoskeleton together) through plakoglobin, desmoplakin, desmogleins, and desmocollins; desmin filaments are connected in a similar way in heart muscle cells.

Plakoglobin

Protein that in humans is encoded by the JUP gene.

A representation of the 3D structure of the protein myoglobin showing turquoise α-helices. This protein was the first to have its structure solved by X-ray crystallography. Toward the right-center among the coils, a prosthetic group called a heme group (shown in gray) with a bound oxygen molecule (red).

Studies investigating the role of plakoglobin in disease pathology have found that suppression of desmoplakin expression by siRNA led to the nuclear localization of plakoglobin, resulting in a reduction in Wnt signaling via Tcf/Lef1 and ensued pathogenesis of ARVC.

Paraneoplastic pemphigus

Autoimmune disorder stemming from an underlying tumor.

Young woman with malar rash, typically found in systemic lupus erythematosus (SLE)

Through immunoprecipitation, target antigens have been found to include desmoglein-3, desmoglein-1, envoplakin, periplakin, desmoplakin 1, desmoplakin 2, and bullous pemphigoid antigen I.

Keratin 1

Member of the keratin family.

Microscopy of keratin filaments inside cells

Keratin 1 has been shown to interact with Desmoplakin and PRKCE.

Plakophilin-2

Protein that in humans is encoded by the PKP2 gene.

A representation of the 3D structure of the protein myoglobin showing turquoise α-helices. This protein was the first to have its structure solved by X-ray crystallography. Toward the right-center among the coils, a prosthetic group called a heme group (shown in gray) with a bound oxygen molecule (red).

Plakophilin 2 localizes to cell desmosomes and nuclei and binds plakoglobin, desmoplakin, and the desmosomal cadherins via N-terminal head domain.

Plakophilin-1

Protein that in humans is encoded by the PKP1 gene.

A representation of the 3D structure of the protein myoglobin showing turquoise α-helices. This protein was the first to have its structure solved by X-ray crystallography. Toward the right-center among the coils, a prosthetic group called a heme group (shown in gray) with a bound oxygen molecule (red).

PKP1 has been shown to interact with Desmoplakin.