Ion channel function of polycystin‐2/polycystin‐1 heteromer revealed by structure‐guided mutagenesis
Sign Up to like & getrecommendations! Published in 2024 at "Febs Letters"
DOI: 10.1002/1873-3468.70059
Abstract: Autosomal-dominant polycystic kidney disease (ADPKD) is caused by mutations affecting polycystin-1 (PC1) or polycystin-2 (PC2). Recent structural data suggest that PC1 and PC2 can form heterotetrameric ion channels with a 3:1 stoichiometry, with the channel… read more here.
Keywords: function; polycystin; ion; pc2 pc1 ... See more keywords
Polycystin‐1 Mutant Alters Mechanotransduction in Response to Collagen and Extracellular Matrix Stiffness via Daam1‐Dependent Microfilament Remodeling
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DOI: 10.1002/advs.202509846
Abstract: Extracellular matrix (ECM) stiffness‐mediated mechanotransduction is a common signaling scheme in both physiological and pathological contexts; however, its molecular mechanisms remain incompletely understood. Polycystin‐1 is a transmembrane protein that is known to participate in mechano‐transduction.… read more here.
Keywords: collagen; microfilament remodeling; extracellular matrix; polycystin ... See more keywords
Polycystic Kidney Disease.
Sign Up to like & getrecommendations! Published in 2017 at "Comprehensive Physiology"
DOI: 10.1002/cphy.c160018
Abstract: Renal cysts, which arise from renal tubules, can be seen in a variety of hereditary and nonhereditary entities. Common mechanisms associated with renal cyst formation include increased cell proliferation, epithelial fluid secretion, and extracellular matrix… read more here.
Keywords: kidney; disease; polycystin; polycystic kidney ... See more keywords
Structure and function of polycystin channels in primary cilia.
Sign Up to like & getrecommendations! Published in 2020 at "Cellular signalling"
DOI: 10.1016/j.cellsig.2020.109626
Abstract: Variants in genes which encode for polycystin-1 and polycystin-2 cause most forms of autosomal dominant polycystic disease (ADPKD). Despite our strong understanding of the genetic determinants of ADPKD, we do not understand the structural features… read more here.
Keywords: channels primary; function polycystin; polycystin; primary cilia ... See more keywords
Glis2 is an early effector of polycystin signaling and a target for therapy in polycystic kidney disease
Sign Up to like & getrecommendations! Published in 2024 at "Nature Communications"
DOI: 10.1038/s41467-024-48025-6
Abstract: Mouse models of autosomal dominant polycystic kidney disease (ADPKD) show that intact primary cilia are required for cyst growth following the inactivation of polycystin-1. The signaling pathways underlying this process, termed cilia-dependent cyst activation (CDCA),… read more here.
Keywords: disease; cyst; kidney disease; polycystic kidney ... See more keywords
Polycystin-1 Regulates Actomyosin Contraction and the Cellular Response to Extracellular Stiffness
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DOI: 10.1038/s41598-019-53061-0
Abstract: Polycystin-1 (PC-1) and 2 (PC-2) are the products of the PKD1 and PKD2 genes, which are mutated in Autosomal Dominant Polycystic Kidney Disease (ADPKD). They form a receptor/channel complex that has been suggested to function… read more here.
Keywords: response extracellular; extracellular stiffness; actomyosin contraction; polycystin ... See more keywords
Polycystin‐1 and hydrostatic pressure are implicated in glioblastoma pathogenesis in vitro
Sign Up to like & getrecommendations! Published in 2022 at "Journal of Cellular and Molecular Medicine"
DOI: 10.1111/jcmm.17212
Abstract: The mechanobiological aspects of glioblastoma (GBM) pathogenesis are largely unknown. Polycystin‐1 (PC1) is a key mechanosensitive protein which perceives extracellular mechanical cues and transforms them into intracellular biochemical signals that elicit a change in cell… read more here.
Keywords: hydrostatic pressure; pc1; gbm; pathogenesis ... See more keywords
Endothelium-Specific Deficiency of Polycystin-1 Promotes Hypertension and Cardiovascular Disorders
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DOI: 10.1161/hypertensionaha.122.19057
Abstract: Background: Autosomal dominant polycystic kidney disease is the most frequent hereditary kidney disease and is generally due to mutations in PKD1 and PKD2, encoding polycystins 1 and 2. In autosomal dominant polycystic kidney disease, hypertension… read more here.
Keywords: hypertension cardiovascular; kidney; polycystin; kidney disease ... See more keywords
A polycystin-2 protein with modified channel properties leads to an increased diameter of renal tubules and to renal cysts
Sign Up to like & getrecommendations! Published in 2021 at "Journal of Cell Science"
DOI: 10.1242/jcs.259013
Abstract: ABSTRACT Mutations in the PKD2 gene cause autosomal-dominant polycystic kidney disease but the physiological role of polycystin-2, the protein product of PKD2, remains elusive. Polycystin-2 belongs to the transient receptor potential (TRP) family of non-selective… read more here.
Keywords: polycystin protein; channel; polycystin; channel properties ... See more keywords
Intraflagellar transport protein 88 interacts with polycystin 2 to regulate mechanosensitive hedgehog signaling in mandibular condylar chondrocytes.
Sign Up to like & getrecommendations! Published in 2022 at "Archives of oral biology"
DOI: 10.2139/ssrn.4045277
Abstract: OBJECTIVE This study aimed to explore whether intraflagellar transport protein 88 (IFT88) was associated with polycystin 2 during mechanotransduction of mandibular condylar chondrocytes. METHODS Rat mandibular condylar chondrocytes isolated from the condylar bone-cartilage junction were… read more here.
Keywords: polycystin; ift88; cyclic tensile; condylar chondrocytes ... See more keywords
The heteromeric PC-1/PC-2 polycystin complex is activated by the PC-1 N-terminus
Sign Up to like & getrecommendations! Published in 2020 at "eLife"
DOI: 10.7554/elife.60684
Abstract: Mutations in the polycystin proteins, PC-1 and PC-2, result in autosomal dominant polycystic kidney disease (ADPKD) and ultimately renal failure. PC-1 and PC-2 enrich on primary cilia, where they are thought to form a heteromeric… read more here.
Keywords: polycystin; polycystin complex; activated terminus; complex activated ... See more keywords