Year: 2020
Type: article
Abstract: Polycystin 2 (PC2 or TRPP1, formerly TRPP2) is a calcium-permeant Transient Receptor Potential (TRP) cation channel expressed primarily on the endoplasmic reticulum (ER) membrane and primary cilia of all cell and tissue types. Despite its ubiquitous expression throughout the body, studies of PC2 have focused primarily on its role in the kidney, as mutations in PC2 lead to the development of autosomal dominant polycystic kidney disease (ADPKD), a debilitating condition for which there is no cure. However, the endogenous role that PC2 plays in the regulation of general cellular homeostasis remains unclear. In this study, we measure how PC2 expression changes in different pathological states, determine that its abundance is increased under conditions of cellular stress in multiple tissues including human disease, and conclude that PC2-deficient cells have increased susceptibility to cell death induced by stress. Our results offer new insight into the normal function of PC2 as a ubiquitous stress-sensitive protein whose expression is up-regulated in response to cell stress to protect against pathological cell death in multiple diseases.
Source: Scientific Reports
Language: English
Subfield: Genetics
Domain: Life Sciences
Open Access status: gold
Estimated APC paid: $1,870
Funders: U.S. Department of Health and Human Services, Yale University, Paul and Daisy Soros Fellowships for New Americans, National Institutes of Health, National Institute of General Medical Sciences, National Institute of Diabetes and Digestive and Kidney Diseases, National Institute of Neurological Disorders and Stroke, National Center for Advancing Translational Sciences
Awards: Tuning neuronal excitability by axonal targeting of Kv7 channels, G1505602298, G1820432668, G2123319225, Center for Polycystic Kidney Disease Research at Yale, G305547285, G3508573990, Institutional National Research Service Award, Super-Resolution Microscopy of Small Quantum Dots to Elucidate the Mechanisms of Alzheimer's Disease, G7341987367, Medical Scientist Training Program, George M. O'Brien Kidney Center at Yale, Regulation of hepatocyte response to chronic liver injury by subcellular calcium, Investigating Polycystin-2 up-regulation as a stress response through its role in non-alcoholic fatty liver disease, G8658498105, A super-resolution microscope for research and education, Yale Liver Center, Yale Clinical and Translational Science Award
OpenAlex ID: W3003551711
