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ARVC: a disease of the desmosome scmr.org |
  A desmosome, also known as macula adherens (plural: maculae adherentes) (Latin for adhering spot), is a cell structure specialized for cell-to-cell adhesion. A type of junctional complex, they are localized spot-like adhesions randomly arranged on the lateral sides of plasma membranes. Desmosomes help to resist shearing forces and are found in simple and stratified squamous epithelium. The intercellular space is very wide (about 30 nm). Desmosomes are also found in muscle tissue where they bind muscles cells to one another.
[edit] StructureDesmosomes are molecular complexes of cell adhesion proteins and linking proteins that attach the cell surface adhesion proteins to intracellular keratin cytoskeletal filaments. The cell adhesion proteins of the desmosome, desmoglein and desmocollin, are members of the cadherin family of cell adhesion molecules. They are transmembrane proteins that bridge the space between adjacent epithelial cells by way of homophilic binding of their extracellular domains to other desmosomal cadherins on the adjacent cell. Both have five extracellular domains, and have calcium-binding motifs. The extracellular domain of the desmosome is called the Extracellular Core Domain (ECD) or the Desmoglea, and is bisected by an electron-dense midline where the desmoglein and desmocollin proteins bind to each other. These proteins can bind in a W, S, or λ manner. On the cytoplasmic side of the plasma membrane, there are two dense structures called the Outer Dense Plaque (ODP) and the Inner Dense Plaque (IDP). These are spanned by the Desmoplakin protein.[1] The Outer Dense Plaque is where the cytoplasmic domains of the cadherins attach to desmoplakin via plakoglobin and plakophillin. The Inner Dense Plaque is where desmoplakin attaches to the intermediate filaments of the cell. [edit] Blistering diseasesIf the connecting adjacent epithelial cells of the skin are not functioning correctly, layers of the skin can pull apart and allow abnormal movements of fluid within the skin, resulting in blisters and other tissue damage. Blistering diseases such as Pemphigus Vulgaris can be due to genetic defects in desmosomal proteins. The symptoms of the disease are thought to be caused by an abnormality in the desmosome-keratin filament complex leading to a breakdown in cell adhesion [edit] HemidesmosomesWhen visualized by electron microscopy, hemidesmosomes appear similar to desmosomes. Rather than linking two cells, hemidesmosomes attach one cell to the extracellular matrix. Rather than using cadherins, hemidesmosomes use integrin cell adhesion proteins. Hemidesmosomes are asymmetrical and are found in epithelial cells, generally connecting the basal aspect of cells of the stratum germinativum to the basement membrane. In relation to teeth, they attach the junctional epithelium to the enamel. [edit] References
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