Tubular obstruction induced polycystin upregulation is pro-fibrotic and induced a severe cystic phenotype in adult mice with autosomal dominant polycystic kidney disease: the coexistence of polycystin loss and gain function in ADPKD

Abstract excerpt

Mutations in PKD1 (encoding polycystin-1) or PKD2 (encoding polycystin-2) gene cause autosomal dominant polycystic kidney disease (ADPKD), however high levels of polycystins are detected in renal tissues of ADPKD patients. Animal studies showed that loss and gain of function of polycystins are both pathogenic and can induce cystic phenotype in the kidney, which are associated with enhanced renal fibrosis. Recent...

Topics

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• Disease Models, Animal
• Disease Progression
• Gene Dosage
• Gene Expression
• Mice
• Mutation
• Polycystic Kidney, Autosomal Dominant
• TRPP Cation Channels

Identifiers and source

Literature Corpus work

62e31405-2f21-5b93-acc2-7d1536719e2b

DOI

10.1101/2021.11.03.467035