Science 8 September 1995: Vol. 269. no. 5229, pp. 1402 - 1406 DOI: 10.1126/science.7660122

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Articles

Neurologic Manifestations of von Hippel-Lindau Disease.

J. A. Butman, W. M. Linehan, and R. R. Lonser (2008)
JAMA 300, 1334-1342
 |  Abstract »  |  Full Text »  |  PDF »

Elongin B/C Recruitment Regulates Substrate Binding by CIS.

J. Piessevaux, L. De Ceuninck, D. Catteeuw, F. Peelman, and J. Tavernier (2008)
J. Biol. Chem. 283, 21334-21346
 |  Abstract »  |  Full Text »  |  PDF »

Allosteric effects in the marginally stable von Hippel-Lindau tumor suppressor protein and allostery-based rescue mutant design.

J. Liu and R. Nussinov (2008)
PNAS 105, 901-906
 |  Abstract »  |  Full Text »  |  PDF »

Pseudohypoxic Pathways in Renal Cell Carcinoma.

G. Bratslavsky, S. Sudarshan, L. Neckers, and W. M. Linehan (2007)
Clin. Cancer Res. 13, 4667-4671
 |  Abstract »  |  Full Text »  |  PDF »

Hypoxia-Inducible Factor Linked to Differential Kidney Cancer Risk Seen with Type 2A and Type 2B VHL Mutations.

L. Li, L. Zhang, X. Zhang, Q. Yan, Y. A. Minamishima, A. F. Olumi, M. Mao, S. Bartz, and W. G. Kaelin Jr. (2007)
Mol. Cell. Biol. 27, 5381-5392
 |  Abstract »  |  Full Text »  |  PDF »

Targeting SUMO E1 to Ubiquitin Ligases: A VIRAL STRATEGY TO COUNTERACT SUMOYLATION.

R. Boggio, A. Passafaro, and S. Chiocca (2007)
J. Biol. Chem. 282, 15376-15382
 |  Abstract »  |  Full Text »  |  PDF »

Identification of the Genes for Kidney Cancer: Opportunity for Disease-Specific Targeted Therapeutics.

W. M. Linehan, P. A. Pinto, R. Srinivasan, M. Merino, P. Choyke, L. Choyke, J. Coleman, J. Toro, G. Glenn, C. Vocke, et al. (2007)
Clin. Cancer Res. 13, 671s-679s
 |  Abstract »  |  Full Text »  |  PDF »

Molecular Biology of Renal Cell Cancer and the Identification of Therapeutic Targets.

O. Iliopoulos (2006)
J. Clin. Oncol. 24, 5593-5600
 |  Abstract »  |  Full Text »  |  PDF »

Requirement of ELC1 for RNA Polymerase II Polyubiquitylation and Degradation in Response to DNA Damage in Saccharomyces cerevisiae..

B. Ribar, L. Prakash, and S. Prakash (2006)
Mol. Cell. Biol. 26, 3999-4005
 |  Abstract »  |  Full Text »  |  PDF »

pVHL Function Is Essential for Endothelial Extracellular Matrix Deposition..

N. Tang, F. Mack, V. H. Haase, M. C. Simon, and R. S. Johnson (2006)
Mol. Cell. Biol. 26, 2519-2530
 |  Abstract »  |  Full Text »  |  PDF »

Novel mutations in FH and expansion of the spectrum of phenotypes expressed in families with hereditary leiomyomatosis and renal cell cancer.

M-H Wei, O Toure, G M Glenn, M Pithukpakorn, L Neckers, C Stolle, P Choyke, R Grubb, L Middelton, M L Turner, et al. (2006)
J. Med. Genet. 43, 18-27
 |  Abstract »  |  Full Text »  |  PDF »

The Ubiquitin-Proteasome Pathway and Its Role in Cancer.

A. Mani and E. P. Gelmann (2005)
J. Clin. Oncol. 23, 4776-4789
 |  Abstract »  |  Full Text »  |  PDF »

Inactivation of the Arylhydrocarbon Receptor Nuclear Translocator (Arnt) Suppresses von Hippel-Lindau Disease-Associated Vascular Tumors in Mice.

E. B. Rankin, D. F. Higgins, J. A. Walisser, R. S. Johnson, C. A. Bradfield, and V. H. Haase (2005)
Mol. Cell. Biol. 25, 3163-3172
 |  Abstract »  |  Full Text »  |  PDF »

ASB2 Is an Elongin BC-interacting Protein That Can Assemble with Cullin 5 and Rbx1 to Reconstitute an E3 Ubiquitin Ligase Complex.

M. L. Heuze, F. C. Guibal, C. A. Banks, J. W. Conaway, R. C. Conaway, Y. E. Cayre, A. Benecke, and P. G. Lutz (2005)
J. Biol. Chem. 280, 5468-5474
 |  Abstract »  |  Full Text »  |  PDF »

The von Hippel-Lindau Tumor Suppressor Protein: Roles in Cancer and Oxygen Sensing.

W.G. KAELIN JR. (2005)
Cold Spring Harb Symp Quant Biol 70, 159-166
 |  Abstract »  |  PDF »

Role of VHL Gene Mutation in Human Cancer.

W. Y. Kim and W. G. Kaelin (2004)
J. Clin. Oncol. 22, 4991-5004
 |  Abstract »  |  Full Text »  |  PDF »

VHL-box and SOCS-box domains determine binding specificity for Cul2-Rbx1 and Cul5-Rbx2 modules of ubiquitin ligases.

T. Kamura, K. Maenaka, S. Kotoshiba, M. Matsumoto, D. Kohda, R. C. Conaway, J. W. Conaway, and K. I. Nakayama (2004)
Genes & Dev. 18, 3055-3065
 |  Abstract »  |  Full Text »  |  PDF »

Both BC-Box Motifs of Adenovirus Protein E4orf6 Are Required To Efficiently Assemble an E3 Ligase Complex That Degrades p53.

P. Blanchette, C. Y. Cheng, Q. Yan, G. Ketner, D. A. Ornelles, T. Dobner, R. C. Conaway, J. W. Conaway, and P. E. Branton (2004)
Mol. Cell. Biol. 24, 9619-9629
 |  Abstract »  |  Full Text »  |  PDF »

Elongation by RNA polymerase II: the short and long of it.

R. J. Sims III, R. Belotserkovskaya, and D. Reinberg (2004)
Genes & Dev. 18, 2437-2468
 |  Abstract »  |  Full Text »  |  PDF »

Genetic Basis of Cancer of the Kidney: Disease-Specific Approaches to Therapy.

W. M. Linehan, J. Vasselli, R. Srinivasan, M. M. Walther, M. Merino, P. Choyke, C. Vocke, L. Schmidt, J. S. Isaacs, G. Glenn, et al. (2004)
Clin. Cancer Res. 10, 6282S-6289S
 |  Abstract »  |  Full Text »  |  PDF »

Asb6, an Adipocyte-specific Ankyrin and SOCS Box Protein, Interacts with APS to Enable Recruitment of Elongins B and C to the Insulin Receptor Signaling Complex.

A. Wilcox, K. D. Katsanakis, F. Bheda, and T. S. Pillay (2004)
J. Biol. Chem. 279, 38881-38888
 |  Abstract »  |  Full Text »  |  PDF »

The von Hippel-Lindau Tumor Suppressor Protein Is a Molten Globule under Native Conditions: IMPLICATIONS FOR ITS PHYSIOLOGICAL ACTIVITIES.

H. Sutovsky and E. Gazit (2004)
J. Biol. Chem. 279, 17190-17196
 |  Abstract »  |  Full Text »  |  PDF »

Tumor Suppressor von Hippel-Lindau (VHL) Stabilization of Jade-1 Protein Occurs through Plant Homeodomains and Is VHL Mutation Dependent.

M. I. Zhou, H. Wang, R. L. Foy, J. J. Ross, and H. T. Cohen (2004)
Cancer Res. 64, 1278-1286
 |  Abstract »  |  Full Text »  |  PDF »

Identification of Jade1, a Gene Encoding a PHD Zinc Finger Protein, in a Gene Trap Mutagenesis Screen for Genes Involved in Anteroposterior Axis Development.

E. Tzouanacou, S. Tweedie, and V. Wilson (2003)
Mol. Cell. Biol. 23, 8553-8552
 |  Abstract »  |  Full Text »  |  PDF »

The von Hippel-Lindau Gene, Kidney Cancer, and Oxygen Sensing.

W. G. Kaelin Jr. (2003)
J. Am. Soc. Nephrol. 14, 2703-2711
 |  Abstract »  |  Full Text »  |  PDF »

Influence of the RNA-Binding Protein HuR in pVHL-Regulated p53 Expression in Renal Carcinoma Cells.

S. Galban, J. L. Martindale, K. Mazan-Mamczarz, I. Lopez de Silanes, J. Fan, W. Wang, J. Decker, and M. Gorospe (2003)
Mol. Cell. Biol. 23, 7083-7095
 |  Abstract »  |  Full Text »  |  PDF »

Pheochromocytoma: The Expanding Genetic Differential Diagnosis.

J. Bryant, J. Farmer, L. J. Kessler, R. R. Townsend, and K. L. Nathanson (2003)
J Natl Cancer Inst 95, 1196-1204
 |  Abstract »  |  Full Text »  |  PDF »

Transcriptional Elongation by RNA Polymerase II and Histone Methylation.

M. Gerber and A. Shilatifard (2003)
J. Biol. Chem. 278, 26303-26306
 |  Abstract »  |  Full Text »  |  PDF »

GABARAP: Lessons for Synaptogenesis.

J. E. Coyle and D. B. Nikolov (2003)
Neuroscientist 9, 205-216
 |  Abstract »  |  PDF »

TIP120A Associates with Cullins and Modulates Ubiquitin Ligase Activity.

K.-W. Min, J.-W. Hwang, J.-S. Lee, Y. Park, T.-a. Tamura, and J.-B. Yoon (2003)
J. Biol. Chem. 278, 15905-15910
 |  Abstract »  |  Full Text »  |  PDF »

Mammalian Elongin A Is Not Essential for Cell Viability but Is Required for Proper Cell Cycle Progression with Limited Alteration of Gene Expression.

K. Yamazaki, T. Aso, Y. Ohnishi, M. Ohno, K. Tamura, T. Shuin, S. Kitajima, and Y. Nakabeppu (2003)
J. Biol. Chem. 278, 13585-13589
 |  Abstract »  |  Full Text »  |  PDF »

von Hippel-Lindau Protein-Mediated Repression of Tumor Necrosis Factor Alpha Translation Revealed through Use of cDNA Arrays.

S. Galban, J. Fan, J. L. Martindale, C. Cheadle, B. Hoffman, M. P. Woods, G. Temeles, J. Brieger, J. Decker, and M. Gorospe (2003)
Mol. Cell. Biol. 23, 2316-2328
 |  Abstract »  |  Full Text »  |  PDF »

von Hippel-Lindau protein binds hyperphosphorylated large subunit of RNA polymerase II through a proline hydroxylation motif and targets it for ubiquitination.

A. V. Kuznetsova, J. Meller, P. O. Schnell, J. A. Nash, M. L. Ignacak, Y. Sanchez, J. W. Conaway, R. C. Conaway, and M. F. Czyzyk-Krzeska (2003)
PNAS 100, 2706-2711
 |  Abstract »  |  Full Text »  |  PDF »

A three-dimensional model of Suppressor Of Cytokine Signalling 1 (SOCS-1).

F. Giordanetto and R. T. Kroemer (2003)
Protein Eng. Des. Sel. 16, 115-124
 |  Abstract »  |  Full Text »  |  PDF »

Eye and Kidney: From Clinical Findings to Genetic Explanations.

H. Izzedine, B. Bodaghi, V. Launay-Vacher, and G. Deray (2003)
J. Am. Soc. Nephrol. 14, 516-529
 |  Full Text »  |  PDF »

Leu-574 of HIF-1alpha Is Essential for the von Hippel-Lindau (VHL)-mediated Degradation Pathway.

L. E. Huang, E. A. Pete, M. Schau, J. Milligan, and J. Gu (2002)
J. Biol. Chem. 277, 41750-41755
 |  Abstract »  |  Full Text »  |  PDF »

The von Hippel-Lindau Tumor Suppressor Stabilizes Novel Plant Homeodomain Protein Jade-1.

M. I. Zhou, H. Wang, J. J. Ross, I. Kuzmin, C. Xu, and H. T. Cohen (2002)
J. Biol. Chem. 277, 39887-39898
 |  Abstract »  |  Full Text »  |  PDF »

Analysis of the Adenovirus E1B-55K-Anchored Proteome Reveals Its Link to Ubiquitination Machinery.

J. N. Harada, A. Shevchenko, A. Shevchenko, D. C. Pallas, and A. J. Berk (2002)
J. Virol. 76, 9194-9206
 |  Abstract »  |  Full Text »  |  PDF »

A Molecular Basis for Stabilization of the von Hippel-Lindau (VHL) Tumor Suppressor Protein by Components of the VHL Ubiquitin Ligase.

T. Kamura, C. S. Brower, R. C. Conaway, and J. W. Conaway (2002)
J. Biol. Chem. 277, 30388-30393
 |  Abstract »  |  Full Text »  |  PDF »

Oxygen-Dependent Ubiquitination and Degradation of Hypoxia-Inducible Factor Requires Nuclear-Cytoplasmic Trafficking of the von Hippel-Lindau Tumor Suppressor Protein.

I. Groulx and S. Lee (2002)
Mol. Cell. Biol. 22, 5319-5336
 |  Abstract »  |  Full Text »  |  PDF »

dELL is an essential RNA polymerase II elongation factor with a general role in development.

J. C. Eissenberg, J. Ma, M. A. Gerber, A. Christensen, J. A. Kennison, and A. Shilatifard (2002)
PNAS 99, 9894-9899
 |  Abstract »  |  Full Text »  |  PDF »

CUL-4A Is Critical for Early Embryonic Development.

B. Li, J. C. Ruiz, and K. T. Chun (2002)
Mol. Cell. Biol. 22, 4997-5005
 |  Abstract »  |  Full Text »  |  PDF »

Identification and Biochemical Characterization of a Novel Transcription Elongation Factor, Elongin A3.

K. Yamazaki, L. Guo, K. Sugahara, C. Zhang, H. Enzan, Y. Nakabeppu, S. Kitajima, and T. Aso (2002)
J. Biol. Chem. 277, 26444-26451
 |  Abstract »  |  Full Text »  |  PDF »

Regulation of Jak2 through the Ubiquitin-Proteasome Pathway Involves Phosphorylation of Jak2 on Y1007 and Interaction with SOCS-1.

D. Ungureanu, P. Saharinen, I. Junttila, D. J. Hilton, and O. Silvennoinen (2002)
Mol. Cell. Biol. 22, 3316-3326
 |  Abstract »  |  Full Text »  |  PDF »

Binding to Elongin C Inhibits Degradation of Interacting Proteins in Yeast.

L. E. Hyman, E. Kwon, S. Ghosh, J. McGee, A. M. B. Chachulska, T. Jackson, and W. H. Baricos (2002)
J. Biol. Chem. 277, 15586-15591
 |  Abstract »  |  Full Text »  |  PDF »

Diverse Effects of Mutations in Exon II of the von Hippel-Lindau (VHL) Tumor Suppressor Gene on the Interaction of pVHL with the Cytosolic Chaperonin and pVHL-Dependent Ubiquitin Ligase Activity.

W. J. Hansen, M. Ohh, J. Moslehi, K. Kondo, W. G. Kaelin, and W. J. Welch (2002)
Mol. Cell. Biol. 22, 1947-1960
 |  Abstract »  |  Full Text »  |  PDF »

Degradation of p53 by adenovirus E4orf6 and E1B55K proteins occurs via a novel mechanism involving a Cullin-containing complex.

E. Querido, P. Blanchette, Q. Yan, T. Kamura, M. Morrison, D. Boivin, W. G. Kaelin, R. C. Conaway, J. W. Conaway, and P. E. Branton (2001)
Genes & Dev. 15, 3104-3117
 |  Abstract »  |  Full Text »  |  PDF »

Molecular Characterization and Ophthalmic Investigation of a Large Family With Type 2A von Hippel-Lindau Disease.

R. C. Allen, A. R. Webster, R. Sui, J. Brown, C. M. Taylor, and E. M. Stone (2001)
Arch Ophthalmol 119, 1659-1665
 |  Abstract »  |  Full Text »  |  PDF »

The Von Hippel-Lindau Protein Interacts with Heteronuclear Ribonucleoprotein A2 and Regulates Its Expression.

P. A. Pioli and W. F. C. Rigby (2001)
J. Biol. Chem. 276, 40346-40352
 |  Abstract »  |  Full Text »  |  PDF »

Molecular Pathogenesis of the Von Hippel-Lindau Hereditary Cancer Syndrome: Implications for Oxygen Sensing.

H. Yang and W. G. Kaelin Jr. (2001)
Cell Growth Differ. 12, 447-455
 |  Full Text »  |  PDF »

Contrasting effects on HIF-1{{alpha}} regulation by disease-causing pVHL mutations correlate with patterns of tumourigenesis in von Hippel-Lindau disease.

S. C. Clifford, M. E. Cockman, A. C. Smallwood, D. R. Mole, E. R. Woodward, P. H. Maxwell, P. J. Ratcliffe, and E. R. Maher (2001)
Hum. Mol. Genet. 10, 1029-1038
 |  Abstract »  |  Full Text »  |  PDF »

Dynamic, Site-specific Interaction of Hypoxia-inducible Factor-1{{alpha}} with the von Hippel-Lindau Tumor Suppressor Protein.

F. Yu, S. B. White, Q. Zhao, and F. S. Lee (2001)
Cancer Res. 61, 4136-4142
 |  Abstract »  |  Full Text »

VHL tumor suppressor regulates Cl-/HCO3- exchange and Na+/H+ exchange activities in renal carcinoma cells.

S. A. KARUMANCHI, L. JIANG, B. KNEBELMANN, A. K. STUART-TILLEY, S. L. ALPER, and V. P. SUKHATME (2001)
Physiol Genomics 5, 119-128
 |  Abstract »  |  Full Text »  |  PDF »

Genotype-phenotype correlation in von Hippel-Lindau syndrome.

C. A. Friedrich (2001)
Hum. Mol. Genet. 10, 763-767
 |  Abstract »  |  Full Text »  |  PDF »

Vascular tumors in livers with targeted inactivation of the von Hippel-Lindau tumor suppressor.

V. H. Haase, J. N. Glickman, M. Socolovsky, and R. Jaenisch (2001)
PNAS 98, 1583-1588
 |  Abstract »  |  Full Text »  |  PDF »

Bcr-Abl kinase down-regulates cyclin-dependent kinase inhibitor p27 in human and murine cell lines.

T. Jonuleit, H. van der Kuip, C. Miething, H. Michels, M. Hallek, J. Duyster, and W. E. Aulitzky (2000)
Blood 96, 1933-1939
 |  Abstract »  |  Full Text »  |  PDF »

Elongin BC complex prevents degradation of von Hippel-Lindau tumor suppressor gene products.

A. R. Schoenfeld, E. J. Davidowitz, and R. D. Burk (2000)
PNAS 97, 8507-8512
 |  Abstract »  |  Full Text »  |  PDF »

Role of chromosome 3p12-p21 tumour suppressor genes in clear cell renal cell carcinoma: analysis of VHL dependent and VHL independent pathways of tumorigenesis.

A Martinez, P Fullwood, K Kondo, T Kishida, M Yao, E R Maher, and F Latif (2000)
Mol. Pathol. 53, 137-144
 |  Abstract »  |  Full Text »

Role of the von Hippel-Lindau Tumor Suppressor Protein during Neuronal Differentiation.

H. Kanno, F. Saljooque, I. Yamamoto, S. Hattori, M. Yao, T. Shuin, and H.-S. U (2000)
Cancer Res. 60, 2820-2824
 |  Abstract »  |  Full Text »  |  PDF »

Elongin from Saccharomyces cerevisiae.

C. M. Koth, M. V. Botuyan, R. J. Moreland, D. B. Jansma, J. W. Conaway, R. C. Conaway, W. J. Chazin, J. D. Friesen, C. H. Arrowsmith, and A. M. Edwards (2000)
J. Biol. Chem. 275, 11174-11180
 |  Abstract »  |  Full Text »  |  PDF »

Requirement for the von Hippel-Lindau Tumor Suppressor Gene for Functional Epidermal Growth Factor Receptor Blockade by Monoclonal Antibody C225 in Renal Cell Carcinoma.

A. D. Perera, E. V. Kleymenova, and C. L. Walker (2000)
Clin. Cancer Res. 6, 1518-1523
 |  Abstract »  |  Full Text »

Ran-mediated Nuclear Export of the von Hippel-Lindau Tumor Suppressor Protein Occurs Independently of Its Assembly with Cullin-2.

I. Groulx, M.-E. Bonicalzi, and S. Lee (2000)
J. Biol. Chem. 275, 8991-9000
 |  Abstract »  |  Full Text »  |  PDF »

Identification and Characterization of Elongin A2, a New Member of the Elongin Family of Transcription Elongation Factors, Specifically Expressed in the Testis.

T. Aso, K. Yamazaki, K. Amimoto, A. Kuroiwa, H. Higashi, Y. Matsuda, S. Kitajima, and M. Hatakeyama (2000)
J. Biol. Chem. 275, 6546-6552
 |  Abstract »  |  Full Text »  |  PDF »

The von Hippel-Lindau Tumor Suppressor Gene Product Promotes, but Is Not Essential for, NEDD8 Conjugation to Cullin-2.

H. Wada, E. T. H. Yeh, and T. Kamitani (1999)
J. Biol. Chem. 274, 36025-36029
 |  Abstract »  |  Full Text »  |  PDF »

The Antiangiogenic Agent Linomide Inhibits the Growth Rate of von Hippel-Lindau Paraganglioma Xenografts to Mice.

D. J. Gross, I. Reibstein, L. Weiss, S. Slavin, I. Stein, M. Neeman, R. Abramovitch, and L. E. Benjamin (1999)
Clin. Cancer Res. 5, 3669-3675
 |  Abstract »  |  Full Text »  |  PDF »

Identification of the von Hippel-Lindau tumor-suppressor protein as part of an active E3 ubiquitin ligase complex.

K. Iwai, K. Yamanaka, T. Kamura, N. Minato, R. C. Conaway, J. W. Conaway, R. D. Klausner, and A. Pause (1999)
PNAS 96, 12436-12441
 |  Abstract »  |  Full Text »  |  PDF »

von Hippel-Lindau Protein Induces Hypoxia-regulated Arrest of Tyrosine Hydroxylase Transcript Elongation in Pheochromocytoma Cells.

S. L. Kroll, W. R. Paulding, P. O. Schnell, M. C. Barton, J.W. Conaway, R. C. Conaway, and M. F. Czyzyk-Krzeska (1999)
J. Biol. Chem. 274, 30109-30114
 |  Abstract »  |  Full Text »  |  PDF »

Differences in Allelic Distribution of Two Polymorphisms in the VHL-Associated Gene CUL2 in Pheochromocytoma Patients without Somatic CUL2 Mutations.

E.-M. Duerr, O. Gimm, D. S. Neuberg, J. B. Kum, S. C. Clifford, S. P. A. Toledo, E. R. Maher, P. L. M. Dahia, and C. Eng (1999)
J. Clin. Endocrinol. Metab. 84, 3207-3211
 |  Abstract »  |  Full Text »

Regulation of the Urokinase-type Plasminogen Activator System by the von Hippel-Lindau Tumor Suppressor Gene.

M. Los, S. Zeamari, J. A. Foekens, M. F. B. G. Gebbink, and E. E. Voest (1999)
Cancer Res. 59, 4440-4445
 |  Abstract »  |  Full Text »  |  PDF »

Studying interactions of four proteins in the yeast two-hybrid system: Structural resemblance of the pVHL/elongin BC/hCUL-2 complex with the ubiquitin ligase complex SKP1/cullin/F-box protein.

A. Pause, B. Peterson, G. Schaffar, R. Stearman, and R. D. Klausner (1999)
PNAS 96, 9533-9538
 |  Abstract »  |  Full Text »  |  PDF »

Binding of elongin A or a von Hippel-Lindau peptide stabilizes the structure of yeast elongin C.

M. V. Botuyan, C. M. Koth, G. Mer, A. Chakrabartty, J. W. Conaway, R. C. Conaway, A. M. Edwards, C. H. Arrowsmith, and W. J. Chazin (1999)
PNAS 96, 9033-9038
 |  Abstract »  |  Full Text »  |  PDF »

The von Hippel-Lindau tumor suppressor protein is a component of an E3 ubiquitin-protein ligase activity.

J. Lisztwan, G. Imbert, C. Wirbelauer, M. Gstaiger, and W. Krek (1999)
Genes & Dev. 13, 1822-1833
 |  Abstract »  |  Full Text »

Physical Interaction and Functional Antagonism between the RNA Polymerase II Elongation Factor ELL and p53.

N. Shinobu, T. Maeda, T. Aso, T. Ito, T. Kondo, K. Koike, and M. Hatakeyama (1999)
J. Biol. Chem. 274, 17003-17010
 |  Abstract »  |  Full Text »  |  PDF »

Conjugation of the ubiquitin-like protein NEDD8 to cullin-2 is linked to von Hippel-Lindau tumor suppressor function.

D. Liakopoulos, T. Busgen, A. Brychzy, S. Jentsch, and A. Pause (1999)
PNAS 96, 5510-5515
 |  Abstract »  |  Full Text »  |  PDF »

The Elongin B Ubiquitin Homology Domain. IDENTIFICATION OF ELONGIN B SEQUENCES IMPORTANT FOR INTERACTION WITH ELONGIN C.

C. S. Brower, A. Shilatifard, T. Mather, T. Kamura, Y. Takagi, D. Haque, A. Treharne, S. I. Foundling, J. W. Conaway, and R. C. Conaway (1999)
J. Biol. Chem. 274, 13629-13636
 |  Abstract »  |  Full Text »  |  PDF »

Rbx1, a Component of the VHL Tumor Suppressor Complex and SCF Ubiquitin Ligase.

T. Kamura, D. M. Koepp, M. N. Conrad, D. Skowyra, R. J. Moreland, O. Iliopoulos, W. S. Lane, W. G. Kaelin Jr., S. J. Elledge, R. C. Conaway, et al. (1999)
Science 284, 657-661
 |  Abstract »  |  Full Text »

Structure of the VHL-ElonginC-ElonginB Complex: Implications for VHL Tumor Suppressor Function.

C. E. Stebbins, W. G. Kaelin Jr., and N. P. Pavletich (1999)
Science 284, 455-461
 |  Abstract »  |  Full Text »

The conserved SOCS box motif in suppressors of cytokine signaling binds to elongins B and C and may couple bound proteins to proteasomal degradation.

J.-G. Zhang, A. Farley, S. E. Nicholson, T. A. Willson, L. M. Zugaro, R. J. Simpson, R. L. Moritz, D. Cary, R. Richardson, G. Hausmann, et al. (1999)
PNAS 96, 2071-2076
 |  Abstract »  |  Full Text »  |  PDF »

Protective Function of von Hippel-Lindau Protein against Impaired Protein Processing in Renal Carcinoma Cells.

M. Gorospe, J. M. Egan, B. Zbar, M. Lerman, L. Geil, I. Kuzmin, and N. J. Holbrook (1999)
Mol. Cell. Biol. 19, 1289-1300
 |  Abstract »  |  Full Text »  |  PDF »

Transcription-Dependent Nuclear-Cytoplasmic Trafficking Is Required for the Function of the von Hippel-Lindau Tumor Suppressor Protein.

S. Lee, M. Neumann, R. Stearman, R. Stauber, A. Pause, G. N. Pavlakis, and R. D. Klausner (1999)
Mol. Cell. Biol. 19, 1486-1497
 |  Abstract »  |  Full Text »  |  PDF »

aHIF: a Natural Antisense Transcript Overexpressed in Human Renal Cancer and During Hypoxia.

C. A. Thrash-Bingham and K. D. Tartof (1999)
J Natl Cancer Inst 91, 143a-151a
 |  Abstract »  |  Full Text »  |  PDF »

The Elongin BC complex interacts with the conserved SOCS-box motif present in members of the SOCS, ras, WD-40 repeat, and ankyrin repeat families.

T. Kamura, S. Sato, D. Haque, L. Liu, W. G. Kaelin Jr., R. C. Conaway, and J. W. Conaway (1998)
Genes & Dev. 12, 3872-3881
 |  Abstract »  |  Full Text »

Factors regulating the transcriptional elongation activity of RNA polymerase II.

A. Shilatifard (1998)
FASEB J 12, 1437-1446
 |  Abstract »  |  Full Text »

Down-regulation of transmembrane carbonic anhydrases in renal cell carcinoma cell lines by wild-type von Hippel-Lindau transgenes.

S. V. Ivanov, I. Kuzmin, M.-H. Wei, S. Pack, L. Geil, B. E. Johnson, E. J. Stanbridge, and M. I. Lerman (1998)
PNAS 95, 12596-12601
 |  Abstract »  |  Full Text »  |  PDF »

Mechanism of Action of RNA Polymerase II Elongation Factor Elongin. MAXIMAL STIMULATION OF ELONGATION REQUIRES CONVERSION OF THE EARLY ELONGATION COMPLEX TO AN ELONGIN-ACTIVABLE FORM.

R. J. Moreland, J. S. Hanas, J. W. Conaway, and R. C. Conaway (1998)
J. Biol. Chem. 273, 26610-26617
 |  Abstract »  |  Full Text »  |  PDF »

pVHL19 is a biologically active product of the von Hippel-Lindau gene arising from internal translation initiation.

O. Iliopoulos, M. Ohh, and W. G. Kaelin Jr. (1998)
PNAS 95, 11661-11666
 |  Abstract »  |  Full Text »  |  PDF »

A second major native von Hippel-Lindau gene product, initiated from an internal translation start site, functions as a tumor suppressor.

A. Schoenfeld, E. J. Davidowitz, and R. D. Burk (1998)
PNAS 95, 8817-8822
 |  Abstract »  |  Full Text »  |  PDF »

Up-Regulation of Vascular Endothelial Growth Factor in Stromal Cells of Hemangioblastomas Is Correlated with Up-Regulation of the Transcription Factor HRF/HIF-2{alpha}.

I. Flamme, M. Krieg, and K. H. Plate (1998)
Am. J. Pathol. 153, 25-29
 |  Abstract »  |  Full Text »  |  PDF »

Identification and Purification of the Holo-ELL Complex. EVIDENCE FOR THE PRESENCE OF ELL-ASSOCIATED PROTEINS THAT SUPPRESS THE TRANSCRIPTIONAL INHIBITORY ACTIVITY OF ELL.

A. Shilatifard (1998)
J. Biol. Chem. 273, 11212-11217
 |  Abstract »  |  Full Text »  |  PDF »

Overexpression of the MEN/ELL Protein, an RNA Polymerase II Elongation Factor, Results in Transformation of Rat1 Cells with Dependence on the Lysine-rich Region.

Y. Kanda, K. Mitani, M. Kurokawa, T. Yamagata, Y. Yazaki, and H. Hirai (1998)
J. Biol. Chem. 273, 5248-5252
 |  Abstract »  |  Full Text »  |  PDF »

The von Hippel-Lindau tumor suppressor gene is required for cell cycle exit upon serum withdrawal.

A. Pause, S. Lee, K. M. Lonergan, and R. D. Klausner (1998)
PNAS 95, 993-998
 |  Abstract »  |  Full Text »  |  PDF »

Regulation of Hypoxia-Inducible mRNAs by the von Hippel-Lindau Tumor Suppressor Protein Requires Binding to Complexes Containing Elongins B/C and Cul2.

K. M. Lonergan, O. Iliopoulos, M. Ohh, T. Kamura, R. C. Conaway, J. W. Conaway, and W. G. Kaelin Jr. (1998)
Mol. Cell. Biol. 18, 732-741
 |  Abstract »  |  Full Text »

Pheochromocytoma in von Hippel-Lindau Disease: Clinical Presentation and Mutation Analysis in a Large, Multigenerational Kindred.

N. O. Atuk, C. Stolle, J. A. Owen Jr., J. T. Carpenter, and M. L. Vance (1998)
J. Clin. Endocrinol. Metab. 83, 117-120
 |  Abstract »  |  Full Text »

Molecular Genetics of Renal Carcinogenesis.

C. Walker (1998)
Toxicol Pathol 26, 113-120
 |  Abstract »  |  PDF »

Formation and Crystallization of Yeast RNA Polymerase II Elongation Complexes.

A. Gnatt, J. Fu, and R. D. Kornberg (1997)
J. Biol. Chem. 272, 30799-30805
 |  Abstract »  |  Full Text »  |  PDF »

Sporadic Pheochromocytomas Are Rarely Associated with Germline Mutations in the vhl Tumor Suppressor Gene or the ret Protooncogene.

H. Brauch, W. Hoeppner, H. Jahnig, T. Wohl, D. Engelhardt, F. Spelsberg, and M. M. Ritter (1997)
J. Clin. Endocrinol. Metab. 82, 4101-4104
 |  Abstract »  |  Full Text »  |  PDF »

Identification of Elongin C Sequences Required for Interaction with the von Hippel-Lindau Tumor Suppressor Protein.

Y. Takagi, A. Pause, R. C. Conaway, and J. W. Conaway (1997)
J. Biol. Chem. 272, 27444-27449
 |  Abstract »  |  Full Text »  |  PDF »

Interaction of Elongation Factors TFIIS and Elongin A with a Human RNA Polymerase II Holoenzyme Capable of Promoter-specific Initiation and Responsive to Transcriptional Activators.

G. Pan, T. Aso, and J. Greenblatt (1997)
J. Biol. Chem. 272, 24563-24571
 |  Abstract »  |  Full Text »  |  PDF »

Structure and Function of RNA Polymerase II Elongation Factor ELL. IDENTIFICATION OF TWO OVERLAPPING ELL FUNCTIONAL DOMAINS THAT GOVERN ITS INTERACTION WITH POLYMERASE AND THE TERNARY ELONGATION COMPLEX.

A. Shilatifard, D. Haque, R. C. Conaway, and J. W. Conaway (1997)
J. Biol. Chem. 272, 22355-22363
 |  Abstract »  |  Full Text »  |  PDF »

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