| Sexually dimorphic renal expression of mouse Klotho is directed by a kidney-specific distal enhancer responsive to HNF1b. | Sexually dimorphic renal expression of mouse Klotho is directed by a kidney-specific distal enhancer responsive to HNF1b.
Jankowski J, Lee HK, Liu C, Wilflingseder J, Hennighausen L., Free PMC Article | 09/23/2024 |
| Functional genomics analysis identifies loss of HNF1B function as a cause of Mayer-Rokitansky-Kuster-Hauser syndrome. | Functional genomics analysis identifies loss of HNF1B function as a cause of Mayer-Rokitansky-Küster-Hauser syndrome.
Thomson E, Tran M, Robevska G, Ayers K, van der Bergen J, Gopalakrishnan Bhaskaran P, Haan E, Cereghini S, Vash-Margita A, Margetts M, Hensley A, Nguyen Q, Sinclair A, Koopman P, Pelosi E., Free PMC Article | 03/11/2023 |
| Transcription factor HNF1beta controls a transcriptional network regulating kidney cell structure and tight junction integrity. | Transcription factor HNF1β controls a transcriptional network regulating kidney cell structure and tight junction integrity.
Tholen LE, Latta F, Martens JHA, Hoenderop JGJ, de Baaij JHF. | 02/4/2023 |
| HNF1beta-associated cyst development and electrolyte disturbances are not explained by BAIAP2L2 expression. | HNF1β-associated cyst development and electrolyte disturbances are not explained by BAIAP2L2 expression.
Tholen LE, Schigt H, Kleuskens SGE, Bos C, Spruijt CG, Willemsen B, Vermeulen M, Hoenderop JGJ, de Baaij JHF. | 12/31/2022 |
| Multiomics analysis reveals that hepatocyte nuclear factor 1beta regulates axon guidance genes in the developing mouse kidney. | Multiomics analysis reveals that hepatocyte nuclear factor 1β regulates axon guidance genes in the developing mouse kidney.
Shao A, Gearhart MD, Chan SC, Miao Z, Susztak K, Igarashi P., Free PMC Article | 10/29/2022 |
| Mechanisms of ion transport regulation by HNF1beta in the kidney: beyond transcriptional regulation of channels and transporters. | Mechanisms of ion transport regulation by HNF1β in the kidney: beyond transcriptional regulation of channels and transporters.
Tholen LE, Hoenderop JGJ, de Baaij JHF., Free PMC Article | 08/6/2022 |
| Hnf1b haploinsufficiency differentially affects developmental target genes in a new renal cysts and diabetes mouse model. | Hnf1b haploinsufficiency differentially affects developmental target genes in a new renal cysts and diabetes mouse model.
Niborski LL, Paces-Fessy M, Ricci P, Bourgeois A, Magalhães P, Kuzma-Kuzniarska M, Lesaulnier C, Reczko M, Declercq E, Zürbig P, Doucet A, Umbhauer M, Cereghini S., Free PMC Article | 02/26/2022 |
| Hepatocyte nuclear factor-1beta shapes the energetic homeostasis of kidney tubule cells. | Hepatocyte nuclear factor-1β shapes the energetic homeostasis of kidney tubule cells.
Piedrafita A, Balayssac S, Casemayou A, Saulnier-Blache JS, Lucas A, Iacovoni JS, Breuil B, Chauveau D, Decramer S, Malet-Martino M, Schanstra JP, Faguer S. | 11/13/2021 |
| Hepatocyte nuclear factor 1beta suppresses canonical Wnt signaling through transcriptional repression of lymphoid enhancer-binding factor 1. | Hepatocyte nuclear factor 1β suppresses canonical Wnt signaling through transcriptional repression of lymphoid enhancer-binding factor 1.
Chan SC, Hajarnis SS, Vrba SM, Patel V, Igarashi P., Free PMC Article | 04/3/2021 |
| Study shows how Hnf1b inactivation in pancreatic ductal cells leads to chronic pancreatitis, neoplasia, and potentiates pancreatic intraepithelial neoplasia formation. This shows a cause of pancreatitis and identifies Hnf1b as a potential tumor suppressor for pancreatic cancer. | Pancreatic Ductal Deletion of Hnf1b Disrupts Exocrine Homeostasis, Leads to Pancreatitis, and Facilitates Tumorigenesis.
Quilichini E, Fabre M, Dirami T, Stedman A, De Vas M, Ozguc O, Pasek RC, Cereghini S, Morillon L, Guerra C, Couvelard A, Gannon M, Haumaitre C., Free PMC Article | 07/25/2020 |
| A function of HNF-1b in the regulation of canonical (beta-catenin-dependent) Wnt signaling in the kidney.HNF-1b and beta-catenin/LEF compete for binding to a beta-catenin/lymphoid enhancer binding factor site overlapping with an HNF-1b half-site. | Hepatocyte nuclear factor-1β regulates Wnt signaling through genome-wide competition with β-catenin/lymphoid enhancer binding factor.
Chan SC, Zhang Y, Pontoglio M, Igarashi P., Free PMC Article | 05/2/2020 |
| Ablation of HNF-1beta in renal epithelial cells leads to the activation of a Twist2-dependent transcriptional network that induces epithelial-mesenchymal transition and aberrant TGF-beta signaling, resulting in renal fibrosis through a cell-nonautonomous mechanism. | Mechanism of Fibrosis in HNF1B-Related Autosomal Dominant Tubulointerstitial Kidney Disease.
Chan SC, Zhang Y, Shao A, Avdulov S, Herrera J, Aboudehen K, Pontoglio M, Igarashi P., Free PMC Article | 09/21/2019 |
| HNF1beta is a transcriptional activator of the CaSR. | Transcription factor HNF1β regulates expression of the calcium-sensing receptor in the thick ascending limb of the kidney.
Kompatscher A, de Baaij JHF, Aboudehen K, Farahani S, van Son LHJ, Milatz S, Himmerkus N, Veenstra GC, Bindels RJM, Hoenderop JGJ. | 07/20/2019 |
| Loss of HNF1B in renal epithelial cells Results in Kidney Disease with Cysts. | Genomic integration of ERRγ-HNF1β regulates renal bioenergetics and prevents chronic kidney disease.
Zhao J, Lupino K, Wilkins BJ, Qiu C, Liu J, Omura Y, Allred AL, McDonald C, Susztak K, Barish GD, Pei L., Free PMC Article | 09/1/2018 |
| HNF1beta renal knockout mouse exhibits down-regulation of Kcnj16.Thus, HNF1beta is a transcriptional activator of Kcnj16. | Loss of transcriptional activation of the potassium channel Kir5.1 by HNF1β drives autosomal dominant tubulointerstitial kidney disease.
Kompatscher A, de Baaij JHF, Aboudehen K, Hoefnagels APWM, Igarashi P, Bindels RJM, Veenstra GJC, Hoenderop JGJ., Free PMC Article | 06/16/2018 |
| Results provide evidence that downregulation of HNF1b and activation of reactive oxygen species/NF-kappaB-mediated in fl ammation play an important role in PCB-153-induced glucose/lipid metabolic disorder. | Polychlorinated biphenyls-153 induces metabolic dysfunction through activation of ROS/NF-κB signaling via downregulation of HNF1b.
Wu H, Yu W, Meng F, Mi J, Peng J, Liu J, Zhang X, Hai C, Wang X., Free PMC Article | 03/24/2018 |
| Hnf1b deletion leads to massively mispatterned ureteric tree network, defective collecting duct differentiation and disrupted tissue architecture, which leads to cystogenesis. This study uncovers a fundamental and recurring role of HNF1B in epithelial organization during early ureteric bud branching and in further patterning and differentiation of the collecting duct system in mouse. | HNF1B controls epithelial organization and cell polarity during ureteric bud branching and collecting duct morphogenesis.
Desgrange A, Heliot C, Skovorodkin I, Akram SU, Heikkilä J, Ronkainen VP, Miinalainen I, Vainio SJ, Cereghini S. | 01/6/2018 |
| This review discusses the role of HNF1B in human and murine pancreas and liver development, summarizes the disease phenotypes and identifies areas for future investigations in HNF1B-associated diabetes and liver disease. [Review Article] | The role of hepatocyte nuclear factor 1β in disease and development.
El-Khairi R, Vallier L. | 11/4/2017 |
| role in osmoregulation in renal collecting ducts | Hepatocyte Nuclear Factor-1β Regulates Urinary Concentration and Response to Hypertonicity.
Aboudehen K, Noureddine L, Cobo-Stark P, Avdulov S, Farahani S, Gearhart MD, Bichet DG, Pontoglio M, Patel V, Igarashi P., Free PMC Article | 10/21/2017 |
| These results demonstrated the crucial role of Hnf1b and Foxa3 in hepatogenesis in vitro and provided a valuable tool for the efficient differentiation of HLCs from ES cells. | Forced expression of Hnf1b/Foxa3 promotes hepatic fate of embryonic stem cells.
Yahoo N, Pournasr B, Rostamzadeh J, Hakhamaneshi MS, Ebadifar A, Fathi F, Baharvand H. | 05/13/2017 |
| HNF-1beta Regulates MicroRNA-200 Expression through a Long Noncoding RNA | Transcription Factor Hepatocyte Nuclear Factor-1β (HNF-1β) Regulates MicroRNA-200 Expression through a Long Noncoding RNA.
Hajarnis SS, Patel V, Aboudehen K, Attanasio M, Cobo-Stark P, Pontoglio M, Igarashi P., Free PMC Article | 01/16/2016 |
| TNFalpha regulates uromodulin expression in a homeostatic setting, but the impact of TNFalpha on uromodulin during kidney injury is superseded by other factors that could inhibit HNF1beta-mediated expression of uromodulin. | The role of tumor necrosis factor alpha in regulating the expression of Tamm-Horsfall Protein (uromodulin) in thick ascending limbs during kidney injury.
Heitmeier M, McCracken R, Micanovic R, Khan S, El-Achkar TM., Free PMC Article | 08/29/2015 |
| Hypoxia induced early up-regulation of Hnf-1beta from 1 to 24 hours, independently of the hypoxia-inducible factor Hif-1alpha. | Hnf-1β transcription factor is an early hif-1α-independent marker of epithelial hypoxia and controls renal repair.
Faguer S, Mayeur N, Casemayou A, Pageaud AL, Courtellemont C, Cartery C, Fournie GJ, Schanstra JP, Tack I, Bascands JL, Chauveau D., Free PMC Article | 01/4/2014 |
| Zyxin regulates migration of renal epithelial cells through activation of hepatocyte nuclear factor-1beta. | Zyxin regulates migration of renal epithelial cells through activation of hepatocyte nuclear factor-1β.
Choi YH, McNally BT, Igarashi P., Free PMC Article | 09/7/2013 |
| Hepatocyte nuclear factor 1beta controls nephron tubular development. | Hepatocyte nuclear factor 1β controls nephron tubular development.
Massa F, Garbay S, Bouvier R, Sugitani Y, Noda T, Gubler MC, Heidet L, Pontoglio M, Fischer E. | 03/30/2013 |