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Paraneoplastic pemphigus (PNP) is an autoimmune blistering disease that is associated with underlying neoplasms. PNP sera reacts with multiple plakin family proteins, among which only envoplakin and periplakin are constantly detected. Periplakin, a membrane-associated precursor of the epidermal cornified envelope and desmosomes, is a member of the plakin family of proteins. The periplakin gene PPL is mapped to human chromosome 16p13.3, and its promoter contains multiple AP2 sites and a SP1 site. Periplakin localizes to desmosomes, the interdesomosomal plasma membrane and intermediate filaments. The periplakin rod domain is necessary for the redistribution of envoplakin to desmosomes and the cytoskeleton by heterodimerization. The periplakin linker domain is required for intermediate filament association. The N-terminus of periplakin accumulates at the cell surface of microvilli in association with cortical actin. Envoplakin and periplakin localize independently to desmosomes, and the distribution of envoplakin at the interdesmosomal plasma membrane depends on heterodimerization with periplakin. The N-terminus of periplakin, therefore, plays an important role in forming the scaffold on which the cornfied envelop is assembled.
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Informations pour la commande
Nom du produit | Ref. Catalogue | COND. | Prix HT | QTÉ | Favoris | |
Anticorps periplakin (AE11) | sc-80605 | 200 µg/ml | $316.00 | |||
periplakin (AE11): m-IgG Fc BP-HRP Kit | sc-539567 | 200 µg Ab; 10 µg BP | $354.00 | |||
periplakin (AE11): m-IgG1 BP-HRP Kit | sc-541562 | 200 µg Ab; 20 µg BP | $354.00 |