Inactivation of integrin-β1 prevents the development of polycystic kidney disease after the loss of polycystin-1

K Lee, S Boctor, LMC Barisoni… - Journal of the American …, 2015 - journals.lww.com
Abstract Dysregulation of polycystin-1 (PC1) leads to autosomal dominant polycystic kidney
disease (ADPKD), a disorder characterized by the formation of multiple bilateral renal cysts,
the progressive accumulation of extracellular matrix (ECM), and the development of
tubulointerstitial fibrosis. Correspondingly, cystic epithelia express higher levels of integrins
(ECM receptors that control various cellular responses, such as cell proliferation, migration,
and survival) that are characteristically altered in cystic cells. To determine whether the …
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