Proteins isolated from the urea-soluble plaque region of desmosomes. Desmoplakin 1 (DSP1, 2871 aa) is found in all desmosomes; DSP-2 is a smaller splice variant found in stratified epithelia. They are long, flexible, rod-like molecules ~100 nm long with two polypeptide chains in parallel. Desmoplakin 3 (plakoglobin) is smaller (745 aa). Autoantibodies against the desmoplakins are important in paraneoplastic pemphigus, a disorder associated with lymphoid malignancies, thymomas, and poorly differentiated sarcomas.
Subjects: Medicine and Health — Chemistry.