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10.15252/emmm.201707532 http://scihub22266oqcxt.onion/10.15252/emmm.201707532 C5666306!5666306!28701330     free     free     free Deprecated: Implicit conversion from float 233.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534 Deprecated: Implicit conversion from float 233.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534 Deprecated: Implicit conversion from float 233.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534       EMBO+Mol+Med 2017 ; 9 (11): 1463-70 Nephropedia Template TP {{tp|p=28701330|t=2017. Oral administration of pyrophosphate inhibits connective tissue calcification |pdf=|usr=}}{{28701330}} gab.com Text Oral administration of pyrophosphate inhibits connective tissue calcification JO: EMBO Mol Med http://www.kidney.de/pget.php?&tw=1&pmid=28701330 #FOAvip Various disorders including pseudoxanthoma elasticum (PXE) and generalized arterial calcification of infancy (GACI), which are caused by inactivating mutations in ABCC6 and ENPP1, respectively, present with extensive tissue calcification due to reduced plasma pyrophosphate (PPi). However, it has always been assumed that the bioavailability of orally administered PPi is negligible. Here, we demonstrate increased PPi concentration in the circulation of humans after oral PPi administration. Furthermore, in mouse models of PXE and GACI, oral PPi provided via drinking water attenuated their ectopic calcification phenotype. Noticeably, provision of drinking water with 0.3 mM PPi to mice heterozygous for inactivating mutations in Enpp1 during pregnancy robustly inhibited ectopic calcification in their Enpp1?/? offspring. Our work shows that orally administered PPi is readily absorbed in humans and mice and inhibits connective tissue calcification in mouse models of PXE and GACI. PPi, which is recognized as safe by the FDA, therefore not only has great potential as an effective and extremely low?cost treatment for these currently intractable genetic disorders, but also in other conditions involving connective tissue calcification. Twit Text FOAVip Oral administration of pyrophosphate inhibits connective tissue calcification ????EMBO Mol Med http://www.kidney.de/pget.php?&tw=1&pmid=28701330 #FOAvip Twit Text # Free Paper on # # # # # LINK http://www.kidney.de/pget.php?&tw=1&pmid=28701330 #FOAvip English Wikipedia <ref>{{Cite pmid|28701330}}</ref> Oral administration of pyrophosphate inhibits connective tissue calcification #MMPMID28701330 Dedinszki D; Szeri F; Kozák E; Pomozi V; T?kési N; Mezei TR; Merczel K; Letavernier E; Tang E; Le Saux O; Arányi T; van de Wetering K; Váradi A EMBO Mol Med 2017[Nov]; 9 (11): 1463-70 PMID28701330show ga Various disorders including pseudoxanthoma elasticum (PXE) and generalized arterial calcification of infancy (GACI), which are caused by inactivating mutations in ABCC6 and ENPP1, respectively, present with extensive tissue calcification due to reduced plasma pyrophosphate (PPi). However, it has always been assumed that the bioavailability of orally administered PPi is negligible. Here, we demonstrate increased PPi concentration in the circulation of humans after oral PPi administration. Furthermore, in mouse models of PXE and GACI, oral PPi provided via drinking water attenuated their ectopic calcification phenotype. Noticeably, provision of drinking water with 0.3 mM PPi to mice heterozygous for inactivating mutations in Enpp1 during pregnancy robustly inhibited ectopic calcification in their Enpp1?/? offspring. Our work shows that orally administered PPi is readily absorbed in humans and mice and inhibits connective tissue calcification in mouse models of PXE and GACI. PPi, which is recognized as safe by the FDA, therefore not only has great potential as an effective and extremely low?cost treatment for these currently intractable genetic disorders, but also in other conditions involving connective tissue calcification. äOral administration of pyrophosphate inhibits connective tissue calcif(epmc).pdf DeepDyve Pubget Overpricing