Article ; Online: Vascular polycystin proteins in health and disease.
Microcirculation (New York, N.Y. : 1994)
2023 Volume 31, Issue 4, Page(s) e12834
Abstract: PKD1 (polycystin 1) and PKD2 (polycystin 2) are expressed in a variety of different cell types, including arterial smooth muscle and endothelial cells. PKD1 is a transmembrane domain protein with a large extracellular N-terminus that is proposed to act ... ...
Abstract | PKD1 (polycystin 1) and PKD2 (polycystin 2) are expressed in a variety of different cell types, including arterial smooth muscle and endothelial cells. PKD1 is a transmembrane domain protein with a large extracellular N-terminus that is proposed to act as a mechanosensor and receptor. PKD2 is a member of the transient receptor potential (TRP) channel superfamily which is also termed TRPP1. Mutations in the genes which encode PKD1 and PKD2 lead to autosomal dominant polycystic kidney disease (ADPKD). ADPKD is one of the most prevalent monogenic disorders in humans and is associated with extrarenal and vascular complications, including hypertension. Recent studies have uncovered mechanisms of activation and physiological functions of PKD1 and PKD2 in arterial smooth muscle and endothelial cells. It has also been found that PKD function is altered in the vasculature during ADPKD and hypertension. We will summarize this work and discuss future possibilities for this area of research. |
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MeSH term(s) | TRPP Cation Channels/metabolism ; TRPP Cation Channels/genetics ; Humans ; Polycystic Kidney, Autosomal Dominant/metabolism ; Polycystic Kidney, Autosomal Dominant/genetics ; Polycystic Kidney, Autosomal Dominant/physiopathology ; Animals ; Muscle, Smooth, Vascular/metabolism ; Hypertension/metabolism ; Hypertension/physiopathology ; Mutation ; Endothelial Cells/metabolism |
Chemical Substances | TRPP Cation Channels ; polycystic kidney disease 1 protein ; polycystic kidney disease 2 protein |
Language | English |
Publishing date | 2023-10-12 |
Publishing country | United States |
Document type | Journal Article ; Review ; Research Support, N.I.H., Extramural ; Research Support, Non-U.S. Gov't |
ZDB-ID | 1217758-1 |
ISSN | 1549-8719 ; 1073-9688 |
ISSN (online) | 1549-8719 |
ISSN | 1073-9688 |
DOI | 10.1111/micc.12834 |
Database | MEDical Literature Analysis and Retrieval System OnLINE |
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