Impaired formation of desmosomal junctions in ADPKD epithelia

Abstract

Mutations in polycystin-1 (PC-1) are responsible for autosomal dominant polycystic kidney disease (ADPKD), characterized by formation of fluid-filled tubular cysts. The PC-1 is a multifunctional protein essential for tubular differentiation and maturation found in desmosomal junctions of epithelial cells where its primary function is to mediate cell–cell… (More)
DOI: 10.1007/s00418-005-0055-3

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