Search Results - "Wetering, Koen"
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Inorganic Pyrophosphate Deficiency Syndromes and Potential Treatments for Pathologic Tissue Calcification
Published in The American journal of pathology (01-05-2022)“…Pathologic soft tissue calcification can occur in both genetic and acquired clinical conditions, causing significant morbidity and mortality. Although the…”
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The Mineralization Regulator ANKH Mediates Cellular Efflux of ATP, Not Pyrophosphate
Published in Journal of bone and mineral research (01-05-2022)“…ABSTRACT The plasma membrane protein ankylosis homologue (ANKH, mouse ortholog: Ank) prevents pathological mineralization of joints by controlling…”
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Generation of fully functional fluorescent fusion proteins to gain insights into ABCC6 biology
Published in FEBS letters (01-03-2021)“…ABCC6 mediates release of ATP from hepatocytes into the blood. Extracellularly, ATP is converted into the mineralization inhibitor pyrophosphate. Consequently,…”
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Multidrug resistance-associated proteins 3, 4, and 5
Published in Pflügers Archiv (01-02-2007)“…We summarize in this paper the recently published results on multidrug resistance-associated proteins 3, 4, and 5 (MRPs 3-5). MRP3 can transport organic…”
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ABCC6–Mediated ATP Secretion by the Liver Is the Main Source of the Mineralization Inhibitor Inorganic Pyrophosphate in the Systemic Circulation—Brief Report
Published in Arteriosclerosis, thrombosis, and vascular biology (01-09-2014)“…OBJECTIVE—Mutations in ABCC6 underlie the ectopic mineralization disorder pseudoxanthoma elasticum (PXE) and some forms of generalized arterial calcification…”
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Oral administration of pyrophosphate inhibits connective tissue calcification
Published in EMBO molecular medicine (01-11-2017)“…Various disorders including pseudoxanthoma elasticum (PXE) and generalized arterial calcification of infancy (GACI), which are caused by inactivating mutations…”
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ABCG2 functions as a general phytoestrogen sulfate transporter in vivo
Published in The FASEB journal (01-10-2012)“…ABCG2 is an ATP‐dependent efflux transporter that limits the systemic exposure of its substrates. The preferred substrates of ABCG2 in vivo are largely…”
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Pyrophosphate Supplementation Prevents Chronic and Acute Calcification in ABCC6-Deficient Mice
Published in The American journal of pathology (01-06-2017)“…Soft tissue calcification occurs in several common acquired pathologies, such as diabetes and hypercholesterolemia, or can result from genetic disorders…”
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Effects of Different Variants in the ENPP1 Gene on the Functional Properties of Ectonucleotide Pyrophosphatase/Phosphodiesterase Family Member 1
Published in Human mutation (01-11-2016)“…ABSTRACT Ectonucleotide pyrophosphatase/phosphodiesterase family member 1 (E‐NPP1), encoded by ENPP1, is a plasma membrane protein that generates inorganic…”
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Comparison of inbred mouse strains shows diverse phenotypic outcomes of intervertebral disc aging
Published in Aging cell (01-05-2020)“…Intervertebral disc degeneration presents a wide spectrum of clinically degenerative disc phenotypes; however, the contribution of genetic background to the…”
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Intestinal breast cancer resistance protein (BCRP)/Bcrp1 and multidrug resistance protein 3 (MRP3)/Mrp3 are involved in the pharmacokinetics of resveratrol
Published in Molecular pharmacology (01-04-2009)“…The phytoestrogen resveratrol has putative health-promoting effects and is present in several dietary constituents. Resveratrol is metabolized extensively in…”
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The pathogenic c.1171A>G (p.Arg391Gly) and c.2359G>A (p.Val787Ile) ABCC6 variants display incomplete penetrance causing pseudoxanthoma elasticum in a subset of individuals
Published in Human mutation (01-12-2022)“…ABCC6 promotes ATP efflux from hepatocytes to bloodstream. ATP is metabolized to pyrophosphate, an inhibitor of ectopic calcification. Pathogenic variants of…”
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Transportomics: screening for substrates of ABC transporters in body fluids using vesicular transport assays
Published in The FASEB journal (01-02-2012)“…ABSTRACT The ATP‐binding cassette (ABC) genes encode the largest family of transmembrane proteins. ABC transporters translocate a wide variety of substrates…”
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Selective induction of chemotherapy resistance of mammary tumors in a conditional mouse model for hereditary breast cancer
Published in Proceedings of the National Academy of Sciences - PNAS (17-07-2007)“…We have studied in vivo responses of "spontaneous" Brca1- and p53-deficient mammary tumors arising in conditional mouse mutants to treatment with doxorubicin,…”
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Negative regulation of urokinase receptor activity by a GPI-specific phospholipase C in breast cancer cells
Published in eLife (29-08-2017)“…The urokinase receptor (uPAR) is a glycosylphosphatidylinositol (GPI)-anchored protein that promotes tissue remodeling, tumor cell adhesion, migration and…”
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MRP1 mediates folate transport and antifolate sensitivity in Plasmodium falciparum
Published in FEBS letters (01-02-2016)“…Multidrug resistance‐associated proteins (MRP) of Plasmodium falciparum have been associated with altered drug sensitivity. Knowledge on MRP substrate…”
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ABCC6 does not transport vitamin K3-glutathione conjugate from the liver: Relevance to pathomechanisms of pseudoxanthoma elasticum
Published in Biochemical and biophysical research communications (25-11-2011)“…► The substrate of ABCC6 preventing soft tissue calcification is not known. ► It was proposed that vitamin K3-glutathione conjugate is the physiological…”
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Contribution of the drug transporter ABCG2 (breast cancer resistance protein) to resistance against anticancer nucleosides
Published in Molecular cancer therapeutics (01-09-2008)“…We have studied the potential contribution of ABCG2 (breast cancer resistance protein) to resistance to nucleoside analogues. In cells transfected with DNA…”
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Mice lacking Mrp3 (Abcc3) have normal bile salt transport, but altered hepatic transport of endogenous glucuronides
Published in Journal of hepatology (01-04-2006)“…Multidrug Resistance Protein 3 (MRP3) transports bile salts and glucuronide conjugates in vitro and is postulated to protect the liver in cholestasis. Whether…”
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Abcc6 Knockout Rat Model Highlights the Role of Liver in PPi Homeostasis in Pseudoxanthoma Elasticum
Published in Journal of investigative dermatology (01-05-2017)“…Pseudoxanthoma elasticum, a heritable ectopic mineralization disorder, is caused by mutations in the ABCC6 gene primarily expressed in the liver and the…”
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