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Search results 1 to 100 out of 502 for Vhl

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Type Details Score
Gene
Type: gene
Organism: human
Gene
Type: gene
Organism: chimpanzee
Gene
Type: gene
Organism: cattle
Gene
Type: gene
Organism: chicken
Gene
Type: gene
Organism: zebrafish
Gene
Type: gene
Organism: frog, western clawed
Gene
Type: gene
Organism: macaque, rhesus
Gene
Type: gene
Organism: rat
Gene
Type: gene
Organism: dog, domestic
Protein Domain
Type: Family
Description: VHL (von Hippel-Lindau disease tumor suppressor) is a component of the VCB (VHL-Elongin BC-CUL2) complex; this complex acts as a ubiquitin-ligase E3 and directs proteasome-dependent degradation of targeted proteins []. Human VHL has been demonstrated to form a ternary complex with elonginB and elonginC proteins []. This complex binds Cul2, which then is involved in regulation of vascular endothelial growth factor mRNA. VHL appears to act as the target recruitment subunit in the E3 ubiquitin ligase complex and recruit hydroxylated hypoxia-inducible factor (HIF) under normal oxygen conditions []. VHL is also involved in transcriptional repression through interaction with HIF1A, HIF1AN and histone deacetylases []. Like human VHL, Drosophila VHL complex containing Cul-2, Rbx1, Elongins B and C, exhibits E3 ubiquitin ligase activity []. However, proteins in this entry also include Von Hippel-Lindau-like (VHLL) protein from human. It may has little or no E3 ubiquitin ligase activity as it lacks the alpha domain required for nucleating the multiprotein E3 ubiquitin ligase complex [].
Protein Coding Gene
Type: protein_coding_gene
Organism: mouse, laboratory
Gene
Type: gene
Organism: human
Gene
Type: gene
Organism: human
Publication
First Author: Stebbins CE
Year: 1999
Journal: Science
Title: Structure of the VHL-ElonginC-ElonginB complex: implications for VHL tumor suppressor function.
Volume: 284
Issue: 5413
Pages: 455-61
Publication
First Author: Qi H
Year: 2004
Journal: Mol Cancer Res
Title: Molecular cloning and characterization of the von Hippel-Lindau-like protein.
Volume: 2
Issue: 1
Pages: 43-52
Protein
Organism: Mus musculus/domesticus
Length: 181  
Fragment?: false
Protein
Organism: Mus musculus/domesticus
Length: 181  
Fragment?: false
Publication
First Author: Hon WC
Year: 2002
Journal: Nature
Title: Structural basis for the recognition of hydroxyproline in HIF-1 alpha by pVHL.
Volume: 417
Issue: 6892
Pages: 975-8
Publication
First Author: Pause A
Year: 1997
Journal: Proc Natl Acad Sci U S A
Title: The von Hippel-Lindau tumor-suppressor gene product forms a stable complex with human CUL-2, a member of the Cdc53 family of proteins.
Volume: 94
Issue: 6
Pages: 2156-61
Publication
First Author: Aso T
Year: 2000
Journal: Biochem Biophys Res Commun
Title: Drosophila von Hippel-Lindau tumor suppressor complex possesses E3 ubiquitin ligase activity.
Volume: 276
Issue: 1
Pages: 355-61
Publication
First Author: Tanimoto K
Year: 2000
Journal: EMBO J
Title: Mechanism of regulation of the hypoxia-inducible factor-1 alpha by the von Hippel-Lindau tumor suppressor protein.
Volume: 19
Issue: 16
Pages: 4298-309
Publication
First Author: Woodward ER
Year: 2000
Journal: Genomics
Title: Comparative sequence analysis of the VHL tumor suppressor gene.
Volume: 65
Issue: 3
Pages: 253-65
Publication
First Author: Thoma CR
Year: 2009
Journal: Nat Cell Biol
Title: VHL loss causes spindle misorientation and chromosome instability.
Volume: 11
Issue: 8
Pages: 994-1001
Publication
First Author: Miró-Murillo M
Year: 2011
Journal: PLoS One
Title: Acute Vhl gene inactivation induces cardiac HIF-dependent erythropoietin gene expression.
Volume: 6
Issue: 7
Pages: e22589
Publication
First Author: Lehmann H
Year: 2015
Journal: J Am Soc Nephrol
Title: Combined Deletion of Vhl and Kif3a Accelerates Renal Cyst Formation.
Volume: 26
Issue: 11
Pages: 2778-88
Publication
First Author: Hell MP
Year: 2014
Journal: Cancer Res
Title: Tumor suppressor VHL functions in the control of mitotic fidelity.
Volume: 74
Issue: 9
Pages: 2422-31
Publication
First Author: Espana-Agusti J
Year: 2017
Journal: Nat Commun
Title: Loss of PBRM1 rescues VHL dependent replication stress to promote renal carcinogenesis.
Volume: 8
Issue: 1
Pages: 2026
Publication
First Author: Zhang W
Year: 2018
Journal: J Exp Med
Title: The E3 ligase VHL controls alveolar macrophage function via metabolic-epigenetic regulation.
Volume: 215
Issue: 12
Pages: 3180-3193
Publication  
First Author: Loots GG
Year: 2018
Journal: Bone
Title: Vhl deficiency in osteocytes produces high bone mass and hematopoietic defects.
Volume: 116
Pages: 307-314
Publication
First Author: Schietke RE
Year: 2012
Journal: PLoS One
Title: Renal tubular HIF-2α expression requires VHL inactivation and causes fibrosis and cysts.
Volume: 7
Issue: 1
Pages: e31034
Publication
First Author: Lee CM
Year: 2009
Journal: Oncogene
Title: VHL Type 2B gene mutation moderates HIF dosage in vitro and in vivo.
Volume: 28
Issue: 14
Pages: 1694-705
Publication
First Author: Haase VH
Year: 2005
Journal: Semin Cell Dev Biol
Title: The VHL tumor suppressor in development and disease: functional studies in mice by conditional gene targeting.
Volume: 16
Issue: 4-5
Pages: 564-74
Publication
First Author: Kapitsinou PP
Year: 2008
Journal: Cell Death Differ
Title: The VHL tumor suppressor and HIF: insights from genetic studies in mice.
Volume: 15
Issue: 4
Pages: 650-9
Publication
First Author: Zhu Y
Year: 2019
Journal: J Exp Med
Title: The E3 ligase VHL promotes follicular helper T cell differentiation via glycolytic-epigenetic control.
Volume: 216
Issue: 7
Pages: 1664-1681
Publication
First Author: Wang Y
Year: 2017
Journal: Mol Carcinog
Title: Neuroprotective effects respond to cerebral ischemia without susceptibility to HB-tumorigenesis in VHL heterozygous knockout mice.
Volume: 56
Issue: 10
Pages: 2342-2351
Publication
First Author: Young AP
Year: 2008
Journal: Nat Cell Biol
Title: VHL loss actuates a HIF-independent senescence programme mediated by Rb and p400.
Volume: 10
Issue: 3
Pages: 361-9
Publication
First Author: Welford SM
Year: 2010
Journal: Mol Cell Biol
Title: Renal oxygenation suppresses VHL loss-induced senescence that is caused by increased sensitivity to oxidative stress.
Volume: 30
Issue: 19
Pages: 4595-603
Publication
First Author: Shen HC
Year: 2009
Journal: PLoS One
Title: Deciphering von Hippel-Lindau (VHL/Vhl)-associated pancreatic manifestations by inactivating Vhl in specific pancreatic cell populations.
Volume: 4
Issue: 4
Pages: e4897
Publication
First Author: Kleymenova E
Year: 2004
Journal: Carcinogenesis
Title: Susceptibility to vascular neoplasms but no increased susceptibility to renal carcinogenesis in Vhl knockout mice.
Volume: 25
Issue: 3
Pages: 309-15
Publication
First Author: Hong SB
Year: 2006
Journal: Lab Invest
Title: Vascular defects and liver damage by the acute inactivation of the VHL gene during mouse embryogenesis.
Volume: 86
Issue: 7
Pages: 664-75
Publication
First Author: Lee JH
Year: 2015
Journal: Immunity
Title: E3 Ubiquitin Ligase VHL Regulates Hypoxia-Inducible Factor-1α to Maintain Regulatory T Cell Stability and Suppressive Capacity.
Volume: 42
Issue: 6
Pages: 1062-74
Publication
First Author: Albers J
Year: 2013
Journal: EMBO Mol Med
Title: Combined mutation of Vhl and Trp53 causes renal cysts and tumours in mice.
Volume: 5
Issue: 6
Pages: 949-64
Publication
First Author: Jiang X
Year: 2013
Journal: J Mol Med (Berl)
Title: Tie2-dependent VHL knockdown promotes airway microvascular regeneration and attenuates invasive growth of Aspergillus fumigatus.
Volume: 91
Issue: 9
Pages: 1081-93
Publication
First Author: Wang SS
Year: 2014
Journal: Proc Natl Acad Sci U S A
Title: Bap1 is essential for kidney function and cooperates with Vhl in renal tumorigenesis.
Volume: 111
Issue: 46
Pages: 16538-43
Publication
First Author: Zanesi N
Year: 2005
Journal: Cancer Res
Title: Lung cancer susceptibility in Fhit-deficient mice is increased by Vhl haploinsufficiency.
Volume: 65
Issue: 15
Pages: 6576-82
Publication
First Author: Seagroves TN
Year: 2010
Journal: Am J Pathol
Title: VHL deletion impairs mammary alveologenesis but is not sufficient for mammary tumorigenesis.
Volume: 176
Issue: 5
Pages: 2269-82
Gene
Type: gene
Organism: chimpanzee
Gene
Type: gene
Organism: macaque, rhesus
Publication
First Author: Iguchi M
Year: 2008
Journal: Nephron Exp Nephrol
Title: Acute inactivation of the VHL gene contributes to protective effects of ischemic preconditioning in the mouse kidney.
Volume: 110
Issue: 3
Pages: e82-90
Publication
First Author: Ma W
Year: 2003
Journal: Cancer Res
Title: Hepatic vascular tumors, angiectasis in multiple organs, and impaired spermatogenesis in mice with conditional inactivation of the VHL gene.
Volume: 63
Issue: 17
Pages: 5320-8
Publication
First Author: Li Q
Year: 2018
Journal: Immunity
Title: E3 Ligase VHL Promotes Group 2 Innate Lymphoid Cell Maturation and Function via Glycolysis Inhibition and Induction of Interleukin-33 Receptor.
Volume: 48
Issue: 2
Pages: 258-270.e5
Publication
First Author: Kim WY
Year: 2006
Journal: EMBO J
Title: Failure to prolyl hydroxylate hypoxia-inducible factor alpha phenocopies VHL inactivation in vivo.
Volume: 25
Issue: 19
Pages: 4650-62
Publication
First Author: Choi D
Year: 2011
Journal: Lab Invest
Title: Vhl is required for normal pancreatic β cell function and the maintenance of β cell mass with age in mice.
Volume: 91
Issue: 4
Pages: 527-38
Publication
First Author: Kurabayashi A
Year: 2013
Journal: PLoS One
Title: Conditional VHL gene deletion causes hypoglycemic death associated with disproportionately increased glucose uptake by hepatocytes through an upregulated IGF-I receptor.
Volume: 8
Issue: 7
Pages: e69139
Publication
First Author: Dirckx N
Year: 2018
Journal: J Clin Invest
Title: Vhl deletion in osteoblasts boosts cellular glycolysis and improves global glucose metabolism.
Volume: 128
Issue: 3
Pages: 1087-1105
Publication
First Author: Mangiavini L
Year: 2014
Journal: Dev Biol
Title: Loss of VHL in mesenchymal progenitors of the limb bud alters multiple steps of endochondral bone development.
Volume: 393
Issue: 1
Pages: 124-36
Publication
First Author: Weng T
Year: 2014
Journal: J Bone Miner Res
Title: Inactivation of Vhl in osteochondral progenitor cells causes high bone mass phenotype and protects against age-related bone loss in adult mice.
Volume: 29
Issue: 4
Pages: 820-9
Publication
First Author: Kuo CY
Year: 2017
Journal: Cancer Res
Title: VHL Inactivation in Precancerous Kidney Cells Induces an Inflammatory Response via ER Stress-Activated IRE1α Signaling.
Volume: 77
Issue: 13
Pages: 3406-3416
Gene
Type: gene
Organism: dog, domestic
Gene
Type: gene
Organism: rat
Gene
Type: gene
Organism: cattle
Gene
Type: gene
Organism: chicken
Gene
Type: gene
Organism: chimpanzee
Gene
Type: gene
Organism: macaque, rhesus
Publication
First Author: Leung JY
Year: 2017
Journal: Cancer Discov
Title: Bap1 and Pbrm1: Determinants of Tumor Grade and mTOR Activation in VHL-Deficient Mouse Models of Renal Cell Carcinoma.
Volume: 7
Issue: 8
Pages: 802-804
Publication
First Author: Russell RC
Year: 2011
Journal: Nat Med
Title: Loss of JAK2 regulation via a heterodimeric VHL-SOCS1 E3 ubiquitin ligase underlies Chuvash polycythemia.
Volume: 17
Issue: 7
Pages: 845-53
Publication
First Author: Kikuchi Y
Year: 1995
Journal: Jpn J Cancer Res
Title: Cloning of the rat homologue of the von Hippel-Lindau tumor suppressor gene and its non-somatic mutation in rat renal cell carcinomas.
Volume: 86
Issue: 10
Pages: 905-9
Publication
First Author: Lolkema MP
Year: 2005
Journal: J Biol Chem
Title: Tumor suppression by the von Hippel-Lindau protein requires phosphorylation of the acidic domain.
Volume: 280
Issue: 23
Pages: 22205-11
Publication
First Author: Izquierdo HM
Year: 2018
Journal: Cell Rep
Title: Von Hippel-Lindau Protein Is Required for Optimal Alveolar Macrophage Terminal Differentiation, Self-Renewal, and Function.
Volume: 24
Issue: 7
Pages: 1738-1746
Publication
First Author: Nargund AM
Year: 2017
Journal: Cell Rep
Title: The SWI/SNF Protein PBRM1 Restrains VHL-Loss-Driven Clear Cell Renal Cell Carcinoma.
Volume: 18
Issue: 12
Pages: 2893-2906
Publication
First Author: Zuo GL
Year: 2015
Journal: PLoS One
Title: Activation of HIFa pathway in mature osteoblasts disrupts the integrity of the osteocyte/canalicular network.
Volume: 10
Issue: 3
Pages: e0121266
Publication
First Author: Frew IJ
Year: 2008
Journal: EMBO J
Title: pVHL and PTEN tumour suppressor proteins cooperatively suppress kidney cyst formation.
Volume: 27
Issue: 12
Pages: 1747-57
Publication
First Author: Takubo K
Year: 2010
Journal: Cell Stem Cell
Title: Regulation of the HIF-1alpha level is essential for hematopoietic stem cells.
Volume: 7
Issue: 3
Pages: 391-402
Publication
First Author: Macías D
Year: 2014
Journal: EMBO Mol Med
Title: Deletion of the von Hippel-Lindau gene causes sympathoadrenal cell death and impairs chemoreceptor-mediated adaptation to hypoxia.
Volume: 6
Issue: 12
Pages: 1577-92
Publication
First Author: Gnarra JR
Year: 1997
Journal: Proc Natl Acad Sci U S A
Title: Defective placental vasculogenesis causes embryonic lethality in VHL-deficient mice.
Volume: 94
Issue: 17
Pages: 9102-7
Publication
First Author: Gao J
Year: 1995
Journal: Cancer Res
Title: Cloning and characterization of a mouse gene with homology to the human von Hippel-Lindau disease tumor suppressor gene: implications for the potential organization of the human von Hippel-Lindau disease gene.
Volume: 55
Issue: 4
Pages: 743-7
Publication
First Author: Frew IJ
Year: 2013
Journal: Eur J Cancer
Title: Genetic deletion of the long isoform of the von Hippel-Lindau tumour suppressor gene product alters microtubule dynamics.
Volume: 49
Issue: 10
Pages: 2433-40
Publication
First Author: Brukamp K
Year: 2007
Journal: Am J Physiol Renal Physiol
Title: Hypoxia and podocyte-specific Vhlh deletion confer risk of glomerular disease.
Volume: 293
Issue: 4
Pages: F1397-407
Publication  
First Author: Johansson E
Year: 2016
Journal: Sci Rep
Title: Simultaneous targeted activation of Notch1 and Vhl-disruption in the kidney proximal epithelial tubular cells in mice.
Volume: 6
Pages: 30739
Publication
First Author: Theilig F
Year: 2011
Journal: Am J Pathol
Title: Tubular deficiency of von Hippel-Lindau attenuates renal disease progression in anti-GBM glomerulonephritis.
Volume: 179
Issue: 5
Pages: 2177-88
Publication
First Author: Kunke M
Year: 2023
Journal: Cell Death Dis
Title: Targeted deletion of von-Hippel-Lindau in the proximal tubule conditions the kidney against early diabetic kidney disease.
Volume: 14
Issue: 8
Pages: 562
Publication
First Author: Wang H
Year: 2018
Journal: Cancer Res
Title: Deletion of the von Hippel-Lindau Gene in Hemangioblasts Causes Hemangioblastoma-like Lesions in Murine Retina.
Volume: 78
Issue: 5
Pages: 1266-1274
Publication
First Author: Puri S
Year: 2009
Journal: Diabetes
Title: A role for von Hippel-Lindau protein in pancreatic beta-cell function.
Volume: 58
Issue: 2
Pages: 433-41
Publication
First Author: Haase VH
Year: 2001
Journal: Proc Natl Acad Sci U S A
Title: Vascular tumors in livers with targeted inactivation of the von Hippel-Lindau tumor suppressor.
Volume: 98
Issue: 4
Pages: 1583-8
Publication
First Author: Hao W
Year: 2023
Journal: Cell Death Dis
Title: Critical role of VHL/BICD2/STAT1 axis in crystal-associated kidney disease.
Volume: 14
Issue: 10
Pages: 680
Publication
First Author: Hsouna A
Year: 2010
Journal: Mol Cell Biol
Title: Drosophila von Hippel-Lindau tumor suppressor gene function in epithelial tubule morphogenesis.
Volume: 30
Issue: 15
Pages: 3779-94
Publication
First Author: Chitrakar A
Year: 2020
Journal: J Immunol
Title: E3 Ubiquitin Ligase Von Hippel-Lindau Protein Promotes Th17 Differentiation.
Volume: 205
Issue: 4
Pages: 1009-1023
Publication
First Author: Hell MP
Year: 2014
Journal: Cancer Res
Title: miR-28-5p promotes chromosomal instability in VHL-associated cancers by inhibiting Mad2 translation.
Volume: 74
Issue: 9
Pages: 2432-43
Publication
First Author: Champion KJ
Year: 2008
Journal: Cancer Res
Title: Endothelial function of von Hippel-Lindau tumor suppressor gene: control of fibroblast growth factor receptor signaling.
Volume: 68
Issue: 12
Pages: 4649-57
Publication
First Author: Zatyka M
Year: 2002
Journal: J Med Genet
Title: Genetic and functional analysis of the von Hippel-Lindau (VHL) tumour suppressor gene promoter.
Volume: 39
Issue: 7
Pages: 463-72
Publication
First Author: Cramer T
Year: 2003
Journal: Cell
Title: HIF-1alpha is essential for myeloid cell-mediated inflammation.
Volume: 112
Issue: 5
Pages: 645-57
Publication
First Author: Ding X
Year: 2020
Journal: Genes Dev
Title: The Daam2-VHL-Nedd4 axis governs developmental and regenerative oligodendrocyte differentiation.
Volume: 34
Issue: 17-18
Pages: 1177-1189
Publication
First Author: Schley G
Year: 2011
Journal: J Am Soc Nephrol
Title: Hypoxia-inducible transcription factors stabilization in the thick ascending limb protects against ischemic acute kidney injury.
Volume: 22
Issue: 11
Pages: 2004-15
Publication  
First Author: Han J
Year: 2022
Journal: J Exp Med
Title: Hypoxia induces adrenomedullin from lung epithelia, stimulating ILC2 inflammation and immunity.
Volume: 219
Issue: 6
Publication
First Author: van Asselt SJ
Year: 2013
Journal: Cilia
Title: Pancreatic cyst development: insights from von Hippel-Lindau disease.
Volume: 2
Issue: 1
Pages: 3
Publication
First Author: Espana-Agusti J
Year: 2016
Journal: PLoS One
Title: Generation and Characterisation of a Pax8-CreERT2 Transgenic Line and a Slc22a6-CreERT2 Knock-In Line for Inducible and Specific Genetic Manipulation of Renal Tubular Epithelial Cells.
Volume: 11
Issue: 2
Pages: e0148055
Publication  
First Author: Yokoe S
Year: 2017
Journal: Int J Mol Sci
Title: Phospholamban Is Downregulated by pVHL-Mediated Degradation through Oxidative Stress in Failing Heart.
Volume: 18
Issue: 11
Publication
First Author: Kessler PM
Year: 1995
Journal: Mol Med
Title: Expression of the Von Hippel-Lindau tumor suppressor gene, VHL, in human fetal kidney and during mouse embryogenesis.
Volume: 1
Issue: 4
Pages: 457-66
Publication
First Author: Bader HL
Year: 2016
Journal: BMC Cancer
Title: Inactivation of the tumor suppressor gene von Hippel-Lindau (VHL) in granulocytes contributes to development of liver hemangiomas in a mouse model.
Volume: 16
Issue: 1
Pages: 797
Publication
First Author: Kurihara T
Year: 2011
Journal: J Cell Biol
Title: Astrocyte pVHL and HIF-α isoforms are required for embryonic-to-adult vascular transition in the eye.
Volume: 195
Issue: 4
Pages: 689-701