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    Vhl von Hippel-Lindau tumor suppressor [ Mus musculus (house mouse) ]

    Gene ID: 22346, updated on 12-May-2024

    GeneRIFs: Gene References Into Functions

    GeneRIFPubMed TitleDate
    VHL loss reprograms the immune landscape to promote an inflammatory myeloid microenvironment in renal tumorigenesis.

    VHL loss reprograms the immune landscape to promote an inflammatory myeloid microenvironment in renal tumorigenesis.
    Wolf MM, Madden MZ, Arner EN, Bader JE, Ye X, Vlach L, Tigue ML, Landis MD, Jonker PB, Hatem Z, Steiner KK, Gaines DK, Reinfeld BI, Hathaway ES, Xin F, Tantawy MN, Haake SM, Jonasch E, Muir A, Weiss VL, Beckermann KE, Rathmell WK, Rathmell JC., Free PMC Article

    04/18/2024
    VHL governs m6A modification and PIK3R3 mRNA stability in clear cell renal cell carcinomas.

    VHL governs m6A modification and PIK3R3 mRNA stability in clear cell renal cell carcinomas.
    Lee H, Zhuang L, Gan B., Free PMC Article

    04/18/2024
    Inhibiting von Hippel-Lindau protein-mediated Dishevelled ubiquitination protects against experimental parkinsonism.

    Inhibiting von Hippel‒Lindau protein-mediated Dishevelled ubiquitination protects against experimental parkinsonism.
    Shen J, Zha Q, Yang QH, Zhou YQ, Liang X, Chen YJ, Qi GX, Zhang XJ, Yao WB, Gao XD, Chen S., Free PMC Article

    04/19/2023
    The von Hippel-Lindau Tumor Suppressor Gene Mutations Modulate Lipocalin-2 Expression in Ferroptotic-Inflammatory Pathways.

    The von Hippel-Lindau Tumor Suppressor Gene Mutations Modulate Lipocalin-2 Expression in Ferroptotic-Inflammatory Pathways.
    Kuo CY, Hsieh PC, Chiu V, Lan CC, Lu KC., Free PMC Article

    02/4/2023
    Deletion of Vhl in Dmp1-Expressing Cells Causes Microenvironmental Impairment of B Cell Lymphopoiesis.

    Deletion of Vhl in Dmp1-Expressing Cells Causes Microenvironmental Impairment of B Cell Lymphopoiesis.
    Chicana B, Abbasizadeh N, Burns C, Taglinao H, Spencer JA, Manilay JO., Free PMC Article

    04/16/2022
    E3 Ubiquitin Ligase Von Hippel-Lindau Protein Promotes Th17 Differentiation.

    E3 Ubiquitin Ligase Von Hippel-Lindau Protein Promotes Th17 Differentiation.
    Chitrakar A, Budda SA, Henderson JG, Axtell RC, Zenewicz LA., Free PMC Article

    03/27/2021
    Rb1/Rbl1/Vhl loss induces mouse subretinal angiomatous proliferation and hemangioblastoma.

    Rb1/Rbl1/Vhl loss induces mouse subretinal angiomatous proliferation and hemangioblastoma.
    Wei R, Ren X, Kong H, Lv Z, Chen Y, Tang Y, Wang Y, Xiao L, Yu T, Hacibekiroglu S, Liang C, Nagy A, Bremner R, Chen D., Free PMC Article

    10/24/2020
    promotes follicular helper T cell differentiation via glycolytic-epigenetic control

    The E3 ligase VHL promotes follicular helper T cell differentiation via glycolytic-epigenetic control.
    Zhu Y, Zhao Y, Zou L, Zhang D, Aki D, Liu YC., Free PMC Article

    06/27/2020
    a novel role for VHL in mediating nephron progenitor differentiation through metabolic regulation, and suggest that VHL is required for normal kidney development.

    Von Hippel-Lindau Acts as a Metabolic Switch Controlling Nephron Progenitor Differentiation.
    Cargill K, Hemker SL, Clugston A, Murali A, Mukherjee E, Liu J, Bushnell D, Bodnar AJ, Saifudeen Z, Ho J, Bates CM, Kostka D, Goetzman ES, Sims-Lucas S., Free PMC Article

    04/25/2020
    Study results in transgenic mice demonstrate that the type 2B (murine G518A representing human R167Q) VHL mutation may influence vascular changes and hemangioblastoma formation by accelerating the maturation of larger-caliber arteries in addition to maintaining and expanding capillary density, which could, in turn, worsen pathological progression via blood perfusion defects.

    Von Hippel-Lindau mutations disrupt vascular patterning and maturation via Notch.
    Arreola A, Payne LB, Julian MH, de Cubas AA, Daniels AB, Taylor S, Zhao H, Darden J, Bautch VL, Rathmell WK, Chappell JC., Free PMC Article

    12/7/2019
    VHL is required for optimal alveolar macrophage terminal differentiation, self-renewal, and function.

    Von Hippel-Lindau Protein Is Required for Optimal Alveolar Macrophage Terminal Differentiation, Self-Renewal, and Function.
    Izquierdo HM, Brandi P, Gómez MJ, Conde-Garrosa R, Priego E, Enamorado M, Martínez-Cano S, Sánchez I, Conejero L, Jimenez-Carretero D, Martín-Puig S, Guilliams M, Sancho D., Free PMC Article

    11/30/2019
    results indicate that VHL acts as a crucial regulatory factor in lung inflammation and fibrosis by regulating alveolar macrophages.

    The E3 ligase VHL controls alveolar macrophage function via metabolic-epigenetic regulation.
    Zhang W, Li Q, Li D, Li J, Aki D, Liu YC., Free PMC Article

    09/7/2019
    Osteocytic Vhl deletion caused high bone mass phenotype, but Hif1a was dispensable in osteocytes.

    Vhl deficiency in osteocytes produces high bone mass and hematopoietic defects.
    Loots GG, Robling AG, Chang JC, Murugesh DK, Bajwa J, Carlisle C, Manilay JO, Wong A, Yellowley CE, Genetos DC.

    07/20/2019
    constitutive or postnatally induced deletion of the hypoxia signaling pathway component von Hippel-Lindau (VHL) in skeletal osteolineage cells of mice led to high bone mass as well as hypoglycemia and increased glucose tolerance, not accounted for by osteocalcin or insulin

    Vhl deletion in osteoblasts boosts cellular glycolysis and improves global glucose metabolism.
    Dirckx N, Tower RJ, Mercken EM, Vangoitsenhoven R, Moreau-Triby C, Breugelmans T, Nefyodova E, Cardoen R, Mathieu C, Van der Schueren B, Confavreux CB, Clemens TL, Maes C., Free PMC Article

    07/20/2019
    Heterozygous mutation in Vhl cooperated with Snf5 deletion to produce hemorrhagic ovarian cysts.

    High Frequency of Ovarian Cyst Development in Vhl(2B/+);Snf5(+/-) Mice.
    Kuwahara Y, Kennedy LM, Karnezis AN, Mora-Blanco EL, Rogers AB, Fletcher CD, Huntsman DG, Roberts CWM, Rathmell WK, Weissman BE., Free PMC Article

    07/6/2019
    Deletion of the von Hippel-Lindau Gene is associated with Hemangioblastoma-like Lesions in Retina.

    Deletion of the von Hippel-Lindau Gene in Hemangioblasts Causes Hemangioblastoma-like Lesions in Murine Retina.
    Wang H, Shepard MJ, Zhang C, Dong L, Walker D, Guedez L, Park S, Wang Y, Chen S, Pang Y, Zhang Q, Gao C, Wong WT, Wiley H, Pacak K, Chew EY, Zhuang Z, Chan CC., Free PMC Article

    01/19/2019
    VHL role in the clear cell renal cell carcinoma

    MYC activation cooperates with Vhl and Ink4a/Arf loss to induce clear cell renal cell carcinoma.
    Bailey ST, Smith AM, Kardos J, Wobker SE, Wilson HL, Krishnan B, Saito R, Lee HJ, Zhang J, Eaton SC, Williams LA, Manocha U, Peters DJ, Pan X, Carroll TJ, Felsher DW, Walter V, Zhang Q, Parker JS, Yeh JJ, Moffitt RA, Leung JY, Kim WY., Free PMC Article

    12/22/2018
    Data show that 100% of von Hippel-Lindau tumor suppressor protein (Vhl) / polybromo 1 protein (Pbrm1) knockout mice developed renal cancers by 20 months of age.

    Loss of PBRM1 rescues VHL dependent replication stress to promote renal carcinogenesis.
    Espana-Agusti J, Warren A, Chew SK, Adams DJ, Matakidou A., Free PMC Article

    10/20/2018
    VHL-HIF-glycolysis axis is essential for the late-stage maturation and function of ILC2s via targeting IL-33-ST2 pathway.

    E3 Ligase VHL Promotes Group 2 Innate Lymphoid Cell Maturation and Function via Glycolysis Inhibition and Induction of Interleukin-33 Receptor.
    Li Q, Li D, Zhang X, Wan Q, Zhang W, Zheng M, Zou L, Elly C, Lee JH, Liu YC., Free PMC Article

    09/22/2018
    Oxidative stress upregulates pVHL expression to induce PLN degradation in failing hearts.

    Phospholamban Is Downregulated by pVHL-Mediated Degradation through Oxidative Stress in Failing Heart.
    Yokoe S, Asahi M., Free PMC Article

    07/7/2018
    Loss of VHL could be driving tumour cell dissemination through stabilization of HIF-1alpha in a murine renal-cell carcinoma model. Overexpression of HIF-1alpha-regulated genes that are upregulated in our VHL knockout cells signifies an aggressive form of clear-cell renal-cell carcinoma.

    CRISPR-Mediated VHL Knockout Generates an Improved Model for Metastatic Renal Cell Carcinoma.
    Schokrpur S, Hu J, Moughon DL, Liu P, Lin LC, Hermann K, Mangul S, Guan W, Pellegrini M, Xu H, Wu L., Free PMC Article

    05/19/2018
    We conditionally targeted Bap1 and Pbrm1 (with Vhl) in the mouse using several Cre drivers.Sglt2 and Villin proximal convoluted tubule drivers failed to cause tumorigenesis, challenging the conventional notion of Clear cell renal cell carcinoma (ccRCC)origins

    Modeling Renal Cell Carcinoma in Mice: Bap1 and Pbrm1 Inactivation Drive Tumor Grade.
    Gu YF, Cohn S, Christie A, McKenzie T, Wolff N, Do QN, Madhuranthakam AJ, Pedrosa I, Wang T, Dey A, Busslinger M, Xie XJ, Hammer RE, McKay RM, Kapur P, Brugarolas J., Free PMC Article

    04/28/2018
    dual inactivation of Vhl with either Bap1 or Pbrm1 results in faithful genetically engineered murine models of clear cell renal cell carcinoma (ccRCC).

    Bap1 and Pbrm1: Determinants of Tumor Grade and mTOR Activation in VHL-Deficient Mouse Models of Renal Cell Carcinoma.
    Leung JY, Kim WY., Free PMC Article

    04/28/2018
    HIF-1alpha-dependent, HIF-2alpha-independent angiogenesis and constitutive diuresis is caused by Vhl deletion in renal epithelia

    Vhl deletion in renal epithelia causes HIF-1α-dependent, HIF-2α-independent angiogenesis and constitutive diuresis.
    Schönenberger D, Rajski M, Harlander S, Frew IJ., Free PMC Article

    03/24/2018
    HIF-1alpha/miR-210 pathway is strongly activated in VHL mutated paragangliomas, weakly activated in SDHx mutated PGLs, and not activated in VHLdel- and SDHxwt/VHLwt-PGLs.

    Role of VHL, HIF1A and SDH on the expression of miR-210: Implications for tumoral pseudo-hypoxic fate.
    Merlo A, Bernardo-Castiñeira C, Sáenz-de-Santa-María I, Pitiot AS, Balbín M, Astudillo A, Valdés N, Scola B, Del Toro R, Méndez-Ferrer S, Piruat JI, Suarez C, Chiara MD., Free PMC Article

    02/24/2018
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