Cilia to basement membrane signalling is a biomechanical driver of autosomal dominant polycystic kidney disease

Abstract excerpt

Autosomal dominant polycystic kidney disease (ADPKD), which affects around 4 million patients worldwide, is characterized by the formation of multiple tubule derived cysts, which grossly enlarge both kidneys and progressively compromise renal function. ADPKD mainly results from mutations in PKD1 , leading to the loss of polycystin-1 protein, which localizes to primary cilia. Primary cilia are required for cyst fo...

Topics

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• Biomechanical Phenomena
• Cell Size
• Cilia
• Kidney
• Mutation
• Polycystic Kidney, Autosomal Dominant
• Polycystic Kidney Diseases
• TRPP Cation Channels

Identifiers and source

Literature Corpus work

d4b2d364-a94c-5e1c-bea7-f13445490e94

DOI

10.1101/2024.06.06.597723