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Cleavage of polycystin-1 requires the receptor for egg jelly domain and is disrupted by human autosomal-dominant polycystic kidney disease 1-associated mutations.

http://www.ncbi.nlm.nih.gov/pubmed/12482949

Polycystin-1 plays an essential role in renal tubular morphogenesis, and disruption of its function causes cystogenesis in human autosomal-dominant polycystic kidney disease (ADPKD). We demonstrated that polycystin-1 undergoes cleavage at G protein coupled receptor proteolytic site in a process that requires the receptor for egg jelly domain. Most of the N-terminal fragment remains tethered at the cell surface, although a small amount is secreted. PKD1-associated mutations in the receptor for egg jelly domain disrupt cleavage, abolish the ability of polycystin-1 to activate signal transducer and activator of transcription-1, and induce tubulogenesis in vitro. We conclude that the cleavage of polycystin-1 is likely essential for its biologic activity.

Pubmed ID: 12482949 RIS Download

Mesh terms: Amino Acid Sequence | Humans | Molecular Sequence Data | Mutagenesis, Site-Directed | Mutation | Polycystic Kidney, Autosomal Dominant | Proteins | Receptors, Cell Surface | Signal Transduction | TRPP Cation Channels

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Associated grants

  • Agency: NIDDK NIH HHS, Id: P50-DK57325

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