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Autosomal dominant polycystic kidney disease (ADPKD) is caused by mutations in the genes encoding either polycystin-1 (PC1) or polycystin-2 (PC2). PC2 acts as a nonselective cation channel and together with PC1 plays a role in intracellular Ca(2+) signaling. Using atomic force microscopy (AFM) imaging, we have shown previously that the N and C termini of(More)
Mutation of polycystin-1 (PC1) is the major cause of autosomal dominant polycystic kidney disease. PC1 has a predicted molecular mass of ~460 kDa comprising a long multidomain extracellular N-terminal region, 11 transmembrane regions, and a short C-terminal region. Because of its size, PC1 has proven difficult to handle biochemically, and structural(More)
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