Desmoplakin Antibody

Desmosomes are intercellular junctions that tightly link adjacent cells. Desmoplakin is an obligate component of functional desmosomes that anchors intermediate filaments to desmosomal plaques. The N-terminus of desmoplakin is required for localization to the desmosome and interacts with the N-terminal region of plakophilin 1 and plakoglobin. The C-terminus of desmoplakin binds with intermediate filaments. In the mid-region of desmoplakin, a coiled-coiled rod domain is responsible for homodimerization. Mutations in DSP, the gene encoding desmoplakin, are the cause of several cardiomyopathies and keratodermas as well as the autoimmune disease paraneoplastic pemphigus [taken from NCBI Entrez Gene (Gene ID: 1832)].
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