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2015 ; 5
(4
): 200-5
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Polycystins and mechanotransduction: From physiology to disease
#MMPMID26618106
Piperi C
; Basdra EK
World J Exp Med
2015[Nov]; 5
(4
): 200-5
PMID26618106
show ga
Polycystins are key mechanosensor proteins able to respond to mechanical forces
of external or internal origin. They are widely expressed in primary cilium and
plasma membrane of several cell types including kidney, vascular endothelial and
smooth muscle cells, osteoblasts and cardiac myocytes modulating their
physiology. Interaction of polycystins with diverse ion channels, cell-cell and
cell-extracellular matrix junctional proteins implicates them in the regulation
of cell structure, mechanical force transmission and mechanotransduction. Their
intracellular localization in endoplasmic reticulum further regulates subcellular
trafficking and calcium homeostasis, finely-tuning overall cellular
mechanosensitivity. Aberrant expression or genetic alterations of polycystins
lead to severe structural and mechanosensing abnormalities including cyst
formation, deregulated flow sensing, aneurysms, defective bone development and
cancer progression, highlighting their vital role in human physiology.