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10.1007/s00424-022-02697-5 http://scihub22266oqcxt.onion/10.1007/s00424-022-02697-5 35554666!9338905!35554666     free     free     free Warning: file_get_contents(https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&id=35554666&cmd=llinks): Failed to open stream: HTTP request failed! HTTP/1.1 429 Too Many Requests in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 215       Pflugers+Arch 2022 ; 474 (8): 901-916 Nephropedia Template TP {{tp|p=35554666|t=2022. Mechanisms of ion transport regulation by HNF1beta in the kidney: beyond transcriptional regulation of channels and transporters |pdf=|usr=}}{{35554666}} gab.com Text Mechanisms of ion transport regulation by HNF1beta in the kidney: beyond transcriptional regulation of channels and transporters JO: Pflugers Arch http://www.kidney.de/pget.php?&tw=1&pmid=35554666 #FOAvip Hepatocyte nuclear factor 1beta (HNF1beta) is a transcription factor essential for the development and function of the kidney. Mutations in and deletions of HNF1beta cause autosomal dominant tubule interstitial kidney disease (ADTKD) subtype HNF1beta, which is characterized by renal cysts, diabetes, genital tract malformations, and neurodevelopmental disorders. Electrolyte disturbances including hypomagnesemia, hyperuricemia, and hypocalciuria are common in patients with ADTKD-HNF1beta. Traditionally, these electrolyte disturbances have been attributed to HNF1beta-mediated transcriptional regulation of gene networks involved in ion transport in the distal part of the nephron including FXYD2, CASR, KCNJ16, and FXR. In this review, we propose additional mechanisms that may contribute to the electrolyte disturbances observed in ADTKD-HNF1beta patients. Firstly, kidney development is severely affected in Hnf1b-deficient mice. HNF1beta is required for nephron segmentation, and the absence of the transcription factor results in rudimentary nephrons lacking mature proximal tubule, loop of Henle, and distal convoluted tubule cluster. In addition, HNF1beta is proposed to be important for apical-basolateral polarity and tight junction integrity in the kidney. Interestingly, cilia formation is unaffected by Hnf1b defects in several models, despite the HNF1beta-mediated transcriptional regulation of many ciliary genes. To what extent impaired nephron segmentation, apical-basolateral polarity, and cilia function contribute to electrolyte disturbances in HNF1beta patients remains elusive. Systematic phenotyping of Hnf1b mouse models and the development of patient-specific kidney organoid models will be essential to advance future HNF1beta research. Twit Text FOAVip Mechanisms of ion transport regulation by HNF1beta in the kidney: beyond transcriptional regulation of channels and transporters ????Pflugers Arch http://www.kidney.de/pget.php?&tw=1&pmid=35554666 #FOAvip Twit Text # Free Paper on # # # # # LINK http://www.kidney.de/pget.php?&tw=1&pmid=35554666 #FOAvip English Wikipedia <ref>{{Cite pmid|35554666}}</ref> Mechanisms of ion transport regulation by HNF1beta in the kidney: beyond transcriptional regulation of channels and transporters #MMPMID35554666 Tholen LE; Hoenderop JGJ; de Baaij JHF Pflugers Arch 2022[Aug]; 474 (8): 901-916 PMID35554666show ga Hepatocyte nuclear factor 1beta (HNF1beta) is a transcription factor essential for the development and function of the kidney. Mutations in and deletions of HNF1beta cause autosomal dominant tubule interstitial kidney disease (ADTKD) subtype HNF1beta, which is characterized by renal cysts, diabetes, genital tract malformations, and neurodevelopmental disorders. Electrolyte disturbances including hypomagnesemia, hyperuricemia, and hypocalciuria are common in patients with ADTKD-HNF1beta. Traditionally, these electrolyte disturbances have been attributed to HNF1beta-mediated transcriptional regulation of gene networks involved in ion transport in the distal part of the nephron including FXYD2, CASR, KCNJ16, and FXR. In this review, we propose additional mechanisms that may contribute to the electrolyte disturbances observed in ADTKD-HNF1beta patients. Firstly, kidney development is severely affected in Hnf1b-deficient mice. HNF1beta is required for nephron segmentation, and the absence of the transcription factor results in rudimentary nephrons lacking mature proximal tubule, loop of Henle, and distal convoluted tubule cluster. In addition, HNF1beta is proposed to be important for apical-basolateral polarity and tight junction integrity in the kidney. Interestingly, cilia formation is unaffected by Hnf1b defects in several models, despite the HNF1beta-mediated transcriptional regulation of many ciliary genes. To what extent impaired nephron segmentation, apical-basolateral polarity, and cilia function contribute to electrolyte disturbances in HNF1beta patients remains elusive. Systematic phenotyping of Hnf1b mouse models and the development of patient-specific kidney organoid models will be essential to advance future HNF1beta research. |*Hepatocyte Nuclear Factor 1-beta/metabolism[MESH] |*Kidney/metabolism[MESH] |*Nephrons/metabolism[MESH] |Animals[MESH] |Electrolytes[MESH] |Ion Transport[MESH] |Membrane Transport Proteins[MESH] |Mice[MESH]Mechanisms of ion transport regulation by HNF1beta in the kidney: beyo(epmc).pdf DeepDyve Pubget Overpricing