UNASSIGNED: High molecular weight @entity10245 transgenic (HMWTg) @entity19 phenocopies the Hyp @entity19 , homolog of @entity1 @entity12145 with hypophosphatemis and abnormal @entity11648 , FGFR, @entity15461 signaling in kidney. Since abnormal Wnt signaling was reported in Hyp @entity19 we assessed whether Wnt signaling was @entity8 and the effect of blocking FGF receptor (FGFR) signaling. Bone mineral density and bone mineral content in female HMWTg @entity19 were significantly reduced. HMWTg @entity19 were gavaged with FGFR inhibitor @entity40617 , or vehicle and were euthanized 24h post treatment. Serum @entity4613 was significantly reduced and urine phosphate was significantly increased in HMWTg and was rescued by @entity40617 . Analysis of kidneys revealed a significant reduction in @entity69973 mRNA in HMWTg that was significantly increased by @entity40617 . Increased @entity10824 , @entity34855 , P-ERK1/2, and decreased @entity69973 protein in HMWTg were rescued by @entity40617 . Wnt inhibitor @entity29827 mRNA and protein was increased in HMWTg and was decreased by @entity40617 . Akt mRNA and protein was decreased in HMWTg and was increased by @entity40617 . The active form of glycogen synthase 3 beta (pGSK3-b) and phosphor-b-catenin were increased in HMWTg and were both decreased by @entity40617 while decreased active-b-catenin in HMWTg was increased by @entity40617 . We conclude that FGFR blockade rescued @entity5886 by regulating FGF and WNT signaling in HMWTg kidneys. This article is protected by copyright. All rights reserved.

FGFR Inhibitor Ameliorates @entity5886 and Impaired XXXX /Wnt Signaling in @entity10245 High Molecular Weight Isoform @entity19 .

@entity1 :: ('9606', 'Species') :: ['human'] @entity12145 :: ('MESH:D053098', 'Disease') :: ['X-linked hypophosphatemic rickets'] @entity8 :: ('MESH:D007674', 'Disease') :: ['impaired in HMWTg kidneys'] @entity40617 :: ('MESH:C568950', 'Chemical') :: ['BGJ398', 'NVP-BGJ398'] @entity34855 :: ('16591(Tax:10090)', 'Gene') :: ['KLOTHO'] @entity10245 :: ('14173(Tax:10090)', 'Gene') :: ['FGF2'] @entity19 :: ('10090', 'Species') :: ['mice', 'mouse'] @entity15461 :: ('9365', 'Gene') :: ['Klotho'] @entity4613 :: ('D010710', 'Chemical') :: ['phosphate'] @entity11648 :: ('8074', 'Gene') :: ['FGF23'] @entity29827 :: ('13798(Tax:10090)', 'Gene') :: ['Engrailed-1'] @entity5886 :: ('MESH:D017674', 'Disease') :: ['hypophosphatemia'] @entity69973 :: ('20505(Tax:10090)', 'Gene') :: ['Npt2a', 'NPT2a'] @entity10824 :: ('14182(Tax:10090)', 'Gene') :: ['FGFR1']
@entity29827 :: (13798(Tax:10090),Gene) :: ['Engrailed-1']
