von Hippel-Lindau disease: genetic and clinical observations.

[1]  Y. Xiong,et al.  The CUL1 C-Terminal Sequence and ROC1 Are Required for Efficient Nuclear Accumulation, NEDD8 Modification, and Ubiquitin Ligase Activity of CUL1 , 2000, Molecular and Cellular Biology.

[2]  R. Conaway,et al.  Activation of HIF1alpha ubiquitination by a reconstituted von Hippel-Lindau (VHL) tumor suppressor complex. , 2000, Proceedings of the National Academy of Sciences of the United States of America.

[3]  Eamonn R. Maher,et al.  Hypoxia Inducible Factor-α Binding and Ubiquitylation by the von Hippel-Lindau Tumor Suppressor Protein* , 2000, The Journal of Biological Chemistry.

[4]  M. V. Heiden,et al.  Redox regulation of p53 during hypoxia , 2000, Oncogene.

[5]  P. Choyke,et al.  Laparoscopic partial adrenalectomy in patients with hereditary forms of pheochromocytoma. , 2000, The Journal of urology.

[6]  M. Ivan,et al.  Ubiquitination of hypoxia-inducible factor requires direct binding to the β-domain of the von Hippel–Lindau protein , 2000, Nature Cell Biology.

[7]  B. Adryan,et al.  Tracheal development and the von Hippel–Lindau tumor suppressor homolog in Drosophila , 2000, Oncogene.

[8]  R. Rottapel,et al.  Suppressor of Cytokine Signaling-1 Inhibits VAV Function through Protein Degradation* , 2000, The Journal of Biological Chemistry.

[9]  K. Goss,et al.  Biology of the adenomatous polyposis coli tumor suppressor. , 2000, Journal of clinical oncology : official journal of the American Society of Clinical Oncology.

[10]  L. Hurst,et al.  Comparative sequence analysis of the VHL tumor suppressor gene. , 2000, Genomics.

[11]  E. Lightcap,et al.  A Nedd8 conjugation pathway is essential for proteolytic targeting of p27Kip1 by ubiquitination. , 2000, Proceedings of the National Academy of Sciences of the United States of America.

[12]  G. Semenza.  HIF-1: mediator of physiological and pathophysiological responses to hypoxia. , 2000, Journal of applied physiology.

[13]  Stephen Lee,et al.  Ran-mediated Nuclear Export of the von Hippel-Lindau Tumor Suppressor Protein Occurs Independently of Its Assembly with Cullin-2* , 2000, The Journal of Biological Chemistry.

[14]  S. Richard,et al.  Central nervous system hemangioblastomas, endolymphatic sac tumors, and von Hippel-Lindau disease , 2000, Neurosurgical Review.

[15]  Z. Ronai,et al.  The SCFHOS/β-TRCP-ROC1 E3 Ubiquitin Ligase Utilizes Two Distinct Domains within CUL1 for Substrate Targeting and Ubiquitin Ligation , 2000, Molecular and Cellular Biology.

[16]  G. Semenza,et al.  Regulation of tumor angiogenesis by p53-induced degradation of hypoxia-inducible factor 1alpha. , 2000, Genes & development.

[17]  J. Pouysségur,et al.  Angiogenesis: how a tumor adapts to hypoxia. , 1999, Biochemical and biophysical research communications.

[18]  S. Gottesman,et al.  Posttranslational quality control: folding, refolding, and degrading proteins. , 1999, Science.

[19]  R. Conaway,et al.  The Rbx1 subunit of SCF and VHL E3 ubiquitin ligase activates Rub1 modification of cullins Cdc53 and Cul2. , 1999, Genes & development.

[20]  D A Hilton,et al.  Overexpression of hypoxia-inducible factor 1alpha in common human cancers and their metastases. , 1999, Cancer research.

[21]  K. Roessler,et al.  Multiple spinal “miliary” hemangioblastomas in von Hippel-Lindau (vHL) disease without cerebellar involvement , 1999, Neurosurgical Review.

[22]  T. Shuin,et al.  Direct interaction of the beta-domain of VHL tumor suppressor protein with the regulatory domain of atypical PKC isotypes. , 1999, Biochemical and biophysical research communications.

[23]  R. Klausner,et al.  The von Hippel-Lindau Tumor Suppressor Gene Inhibits Hepatocyte Growth Factor/Scatter Factor-Induced Invasion and Branching Morphogenesis in Renal Carcinoma Cells , 1999, Molecular and Cellular Biology.

[24]  T. Stearns,et al.  Components of an SCF ubiquitin ligase localize to the centrosome and regulate the centrosome duplication cycle. , 1999, Genes & development.

[25]  H. Neumann,et al.  Preserved adrenocortical function after laparoscopic bilateral adrenal sparing surgery for hereditary pheochromocytoma. , 1999, The Journal of clinical endocrinology and metabolism.

[26]  Michele Pagano,et al.  SKP2 is required for ubiquitin-mediated degradation of the CDK inhibitor p27 , 1999, Nature Cell Biology.

[27]  Wilhelm Krek,et al.  p45SKP2 promotes p27Kip1 degradation and induces S phase in quiescent cells , 1999, Nature Cell Biology.

[28]  M. Gstaiger,et al.  The von Hippel-Lindau tumor suppressor protein is a component of an E3 ubiquitin-protein ligase activity. , 1999, Genes & development.

[29]  W. Linehan,et al.  Plasma normetanephrine and metanephrine for detecting pheochromocytoma in von Hippel-Lindau disease and multiple endocrine neoplasia type 2. , 1999, The New England journal of medicine.

[30]  K Nasmyth,et al.  Cdc53/cullin and the essential Hrt1 RING-H2 subunit of SCF define a ubiquitin ligase module that activates the E2 enzyme Cdc34. , 1999, Genes & development.

[31]  C. Wykoff,et al.  The tumour suppressor protein VHL targets hypoxia-inducible factors for oxygen-dependent proteolysis , 1999, Nature.

[32]  H. Brauch,et al.  Trichloroethylene exposure and specific somatic mutations in patients with renal cell carcinoma. , 1999, Journal of the National Cancer Institute.

[33]  R. Kaufman,et al.  Stress signaling from the lumen of the endoplasmic reticulum: coordination of gene transcriptional and translational controls. , 1999, Genes & development.

[34]  V. Sukhatme,et al.  Transforming Growth Factor β1 Is a Target for the von Hippel-Lindau Tumor Suppressor and a Critical Growth Factor for Clear Cell Renal Carcinoma , 1999 .

[35]  M. Scheffner,et al.  Interaction between ubiquitin–protein ligase SCFSKP2 and E2F-1 underlies the regulation of E2F-1 degradation , 1999, Nature Cell Biology.

[36]  S. Elledge,et al.  Rbx1, a component of the VHL tumor suppressor complex and SCF ubiquitin ligase. , 1999, Science.

[37]  W. Kaelin,et al.  Structure of the VHL-ElonginC-ElonginB complex: implications for VHL tumor suppressor function. , 1999, Science.

[38]  E. Yeh,et al.  Identification of NEDD8-conjugation site in human cullin-2. , 1999, Biochemical and biophysical research communications.

[39]  Z. Ronai,et al.  Recruitment of a ROC1–CUL1 Ubiquitin Ligase by Skp1 and HOS to Catalyze the Ubiquitination of IκBα , 1999 .

[40]  H. Zhu,et al.  Oxygen sensing and signaling: impact on the regulation of physiologically important genes. , 1999, Respiration physiology.

[41]  B. Seizinger,et al.  Alternate choice of initiation codon produces a biologically active product of the von Hippel Lindau gene with tumor suppressor activity , 1999, Oncogene.

[42]  R. Klausner,et al.  Transcription-Dependent Nuclear-Cytoplasmic Trafficking Is Required for the Function of the von Hippel-Lindau Tumor Suppressor Protein , 1999, Molecular and Cellular Biology.

[43]  M. Gorospe,et al.  Protective Function of von Hippel-Lindau Protein against Impaired Protein Processing in Renal Carcinoma Cells , 1999, Molecular and Cellular Biology.

[44]  G. Semenza,et al.  Regulation of mammalian O2 homeostasis by hypoxia-inducible factor 1. , 1999, Annual review of cell and developmental biology.

[45]  E. Ruoslahti,et al.  Fibronectin and its integrin receptors in cancer. , 1999, Advances in cancer research.

[46]  R. Deshaies.  SCF and Cullin/Ring H2-based ubiquitin ligases. , 1999, Annual review of cell and developmental biology.

[47]  R. Conaway,et al.  Transcription elongation and human disease. , 1999, Annual review of biochemistry.

[48]  J. Kere,et al.  Long-Term Prognosis of Haemangioblastoma of the CNS: Impact of von Hippel-Lindau Disease , 1999, Acta Neurochirurgica.

[49]  W. Kaelin,et al.  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. , 1998, Genes & development.

[50]  M. Mann,et al.  Identification of the receptor component of the IκBα–ubiquitin ligase , 1998, Nature.

[51]  K. Plate,et al.  Coexpression of erythropoietin and vascular endothelial growth factor in nervous system tumors associated with von Hippel-Lindau tumor suppressor gene loss of function , 1998 .

[52]  M. Wei,et al.  Down-regulation of transmembrane carbonic anhydrases in renal cell carcinoma cell lines by wild-type von Hippel-Lindau transgenes. , 1998, Proceedings of the National Academy of Sciences of the United States of America.

[53]  D. Mukhopadhyay,et al.  Activation of Sp1-mediated Vascular Permeability Factor/Vascular Endothelial Growth Factor Transcription Requires Specific Interaction with Protein Kinase C ζ* , 1998, The Journal of Biological Chemistry.

[54]  B. Scheithauer,et al.  Peripheral nerve hemangioblastoma. , 1998, Modern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc.

[55]  R. Sutton,et al.  Allelotype analysis of oesophageal adenocarcinoma: loss of heterozygosity occurs at multiple sites. , 1998, British Journal of Cancer.

[56]  W. Kaelin,et al.  pVHL19 is a biologically active product of the von Hippel-Lindau gene arising from internal translation initiation. , 1998, Proceedings of the National Academy of Sciences of the United States of America.

[57]  J. Gervais,et al.  Human CUL-1 associates with the SKP1/SKP2 complex and regulates p21(CIP1/WAF1) and cyclin D proteins. , 1998, Proceedings of the National Academy of Sciences of the United States of America.

[58]  R. Burk,et al.  A second major native von Hippel-Lindau gene product, initiated from an internal translation start site, functions as a tumor suppressor. , 1998, Proceedings of the National Academy of Sciences of the United States of America.

[59]  L. Huang,et al.  Regulation of hypoxia-inducible factor 1α is mediated by an O2-dependent degradation domain via the ubiquitin-proteasome pathway , 1998 .

[60]  S. Libutti,et al.  Multiple neuroendocrine tumors of the pancreas in von Hippel-Lindau disease patients: histopathological and molecular genetic analysis. , 1998, The American journal of pathology.

[61]  J. Adler,et al.  Treatment of hemangioblastomas in von Hippel-Lindau disease with linear accelerator-based radiosurgery. , 1998, Neurosurgery.

[62]  R. Deshaies,et al.  Human CUL1 forms an evolutionarily conserved ubiquitin ligase complex (SCF) with SKP1 and an F-box protein. , 1998, Proceedings of the National Academy of Sciences of the United States of America.

[63]  Campello,et al.  Haemangioblastoma of the central nervous system in von Hippel–Lindau disease , 1998, Journal of internal medicine.

[64]  H. Neumann,et al.  Genotype–phenotype correlations in von hippel–lindau disease , 1998, Journal of internal medicine.

[65]  Goldfarb.  Nephron‐sparing surgery and renal transplantation in patients with renal cell carcinoma and von Hippel–Lindau disease , 1998, Journal of internal medicine.

[66]  Lips,et al.  The eye in von Hippel–Lindau disease. Long‐term follow‐up of screening and treatment: recommendations , 1998, Journal of internal medicine.

[67]  D. Louis,et al.  The von Hippel-Lindau tumor suppressor protein is required for proper assembly of an extracellular fibronectin matrix. , 1998, Molecular cell.

[68]  K. Kinzler,et al.  Landscaping the Cancer Terrain , 1998, Science.

[69]  R. Conaway,et al.  The Elongin BC complex and the von Hippel-Lindau tumor suppressor protein. , 1998, Biochimica et biophysica acta.

[70]  M. Goebl,et al.  Modification of yeast Cdc53p by the ubiquitin-related protein rub1p affects function of the SCFCdc4 complex. , 1998, Genes & development.

[71]  K Nasmyth,et al.  Mass spectrometric analysis of the anaphase-promoting complex from yeast: identification of a subunit related to cullins. , 1998, Science.

[72]  R. Klausner,et al.  The von Hippel-Lindau tumor suppressor gene is required for cell cycle exit upon serum withdrawal. , 1998, Proceedings of the National Academy of Sciences of the United States of America.

[73]  W. Linehan,et al.  Loss of heterozygosity and somatic mutations of the VHL tumor suppressor gene in sporadic cerebellar hemangioblastomas. , 1998, Cancer research.

[74]  W. Kaelin,et al.  Regulation of Hypoxia-Inducible mRNAs by the von Hippel-Lindau Tumor Suppressor Protein Requires Binding to Complexes Containing Elongins B/C and Cul2 , 1998, Molecular and Cellular Biology.

[75]  J. Massagué.  TGF-beta signal transduction. , 1998, Annual review of biochemistry.

[76]  W. Linehan,et al.  Improved detection of germline mutations in the von Hippel‐Lindau disease tumor suppressor gene , 1998, Human mutation.

[77]  H. Riezman.  The Ins and Outs of Protein Translocation , 1997, Science.

[78]  A. Bardelli,et al.  "Invasive-growth" signaling by the Met/HGF receptor: the hereditary renal carcinoma connection. , 1997, Biochimica et biophysica acta.

[79]  D. Mukhopadhyay,et al.  The von Hippel-Lindau Gene Product Inhibits Vascular Permeability Factor/Vascular Endothelial Growth Factor Expression in Renal Cell Carcinoma by Blocking Protein Kinase C Pathways* , 1997, The Journal of Biological Chemistry.

[80]  A. Vortmeyer,et al.  Allelic deletion and mutation of the von Hippel-Lindau (VHL) tumor suppressor gene in pancreatic microcystic adenomas. , 1997, The American journal of pathology.

[81]  H. Neumann,et al.  Functioning thoracic paraganglioma: association with Von Hippel-Lindau syndrome. , 1997, The Journal of clinical endocrinology and metabolism.

[82]  L. Mechtler,et al.  Spinal Leptomeningeal Hemangioblastomatosis in von Hippel‐Lindau Disease: Magnetic Resonance and Pathological Findings , 1997, Journal of neuroimaging : official journal of the American Society of Neuroimaging.

[83]  D. Mukhopadhyay,et al.  The von Hippel-Lindau tumor suppressor gene product interacts with Sp1 to repress vascular endothelial growth factor promoter activity , 1997, Molecular and cellular biology.

[84]  B. Ponder,et al.  Genetic predisposition to phaeochromocytoma: analysis of candidate genes GDNF, RET and VHL. , 1997, Human molecular genetics.

[85]  G. M. Glenn,et al.  Epididymal cystadenomas in von Hippel-Lindau disease. , 1997, Urology.

[86]  M. Emmert-Buck,et al.  von Hippel-Lindau gene deletion detected in the stromal cell component of a cerebellar hemangioblastoma associated with von Hippel-Lindau disease. , 1997, Human pathology.

[87]  W. Kaelin,et al.  The Molecular Basis of Von Hippel-Lindau Disease , 1997, Molecular medicine.

[88]  S. Richard,et al.  Somatic inactivation of the VHL gene in Von Hippel-Lindau disease tumors. , 1997, American journal of human genetics.

[89]  W. Poon,et al.  Molecular genetic analysis of the von Hippel-Lindau disease tumor suppressor gene in familial and sporadic cerebellar hemangioblastomas. , 1997, American journal of clinical pathology.

[90]  A. Kibel,et al.  Immunostaining of the von Hippel-Lindau gene product in normal and neoplastic human tissues. , 1997, Human pathology.

[91]  W. Kaelin,et al.  Regulation of vascular endothelial growth factor by hypoxia and its modulation by the von Hippel-Lindau tumor suppressor gene. , 1997, Kidney international.

[92]  J. Goldblatt,et al.  Variable penetrance of familial pheochromocytoma associated with the von Hippel Lindau gene mutation, S68W , 1997 .

[93]  P. Choyke,et al.  Endolymphatic sac tumors. A source of morbid hearing loss in von Hippel-Lindau disease. , 1997, JAMA.

[94]  M. Tan,et al.  Phaeochromocytoma: a ten-year survey. , 1997, QJM : monthly journal of the Association of Physicians.

[95]  D. Livingston,et al.  Activation of Hypoxia-inducible Transcription Factor Depends Primarily upon Redox-sensitive Stabilization of Its α Subunit* , 1996, The Journal of Biological Chemistry.

[96]  W. Linehan,et al.  Allelic deletions of the VHL gene detected in multiple microscopic clear cell renal lesions in von Hippel-Lindau disease patients. , 1996, The American journal of pathology.

[97]  F. Duh,et al.  Isolation and characterization of the full-length 3′ untranslated region of the human von Hippel-Lindau tumor suppressor gene , 1996, Human Genetics.

[98]  Y. Miyagi,et al.  VON HIPPEL–LINDAU TUMOUR SUPPRESSOR GENE. LOCALIZATION OF EXPRESSION BY IN SITU HYBRIDIZATION , 1996, The Journal of pathology.

[99]  Virgil L. Woods,et al.  A polymeric form of fibronectin has antimetastatic effects against multiple tumor types , 1996, Nature Medicine.

[100]  K. Kinzler,et al.  Lessons from Hereditary Colorectal Cancer , 1996, Cell.

[101]  R. Conaway,et al.  Characterization of Elongin C Functional Domains Required for Interaction with Elongin B and Activation of Elongin A* , 1996, The Journal of Biological Chemistry.

[102]  M. Goldberg,et al.  Hypoxia-inducible Protein Binding to Vascular Endothelial Growth Factor mRNA and Its Modulation by the von Hippel-Lindau Protein* , 1996, The Journal of Biological Chemistry.

[103]  R. Conaway,et al.  The inducible elongin A elongation activation domain: structure, function and interaction with the elongin BC complex. , 1996, The EMBO journal.

[104]  R. Klausner,et al.  Post-transcriptional regulation of vascular endothelial growth factor mRNA by the product of the VHL tumor suppressor gene. , 1996, Proceedings of the National Academy of Sciences of the United States of America.

[105]  W. Kaelin,et al.  Negative regulation of hypoxia-inducible genes by the von Hippel-Lindau protein. , 1996, Proceedings of the National Academy of Sciences of the United States of America.

[106]  J. Jääskeläinen,et al.  Gamma knife radiosurgery in 11 hemangioblastomas. , 1996, Journal of neurosurgery.

[107]  K. Plate,et al.  Pathology, genetics and cell biology of hemangioblastomas. , 1996, Histology and histopathology.

[108]  P. Jackson.  Cell cycle: Cull and destroy , 1996, Current Biology.

[109]  G. Semenza,et al.  Activation of vascular endothelial growth factor gene transcription by hypoxia-inducible factor 1 , 1996, Molecular and cellular biology.

[110]  R. Hofstra,et al.  Extensive mutation scanning of RET in sporadic medullary thyroid carcinoma and of RET and VHL in sporadic pheochromocytoma reveals involvement of these genes in only a minority of cases. , 1996, The Journal of clinical endocrinology and metabolism.

[111]  F. Chen,et al.  Genotype-phenotype correlation in von Hippel-Lindau disease: identification of a mutation associated with VHL type 2A. , 1996, Journal of medical genetics.

[112]  H. Höfler,et al.  Mutations in the VHL tumor suppressor gene and associated lesions in families with von Hippel-Lindau disease from central Europe , 1996, Human Genetics.

[113]  Stephen J. Elledge,et al.  SKP1 Connects Cell Cycle Regulators to the Ubiquitin Proteolysis Machinery through a Novel Motif, the F-Box , 1996, Cell.

[114]  Edward T Kipreos,et al.  cul-1 Is Required for Cell Cycle Exit in C. elegans and Identifies a Novel Gene Family , 1996, Cell.

[115]  G. Reifenberger,et al.  MUTATION OF THE VON HIPPEL–LINDAU TUMOUR SUPPRESSOR GENE IN CAPILLARY HAEMANGIOBLASTOMAS OF THE CENTRAL NERVOUS SYSTEM , 1996, The Journal of pathology.

[116]  D. Kondziolka,et al.  Radiosurgery for hemangioblastoma: results of a multiinstitutional experience. , 1996, International journal of radiation oncology, biology, physics.

[117]  P. Schofield,et al.  Expression of the von Hippel-Lindau disease tumour suppressor gene during human embryogenesis. , 1996, Human molecular genetics.

[118]  R. Klausner,et al.  Molecular cloning of the von Hippel-Lindau tumor suppressor gene and its role in renal carcinoma. , 1996, Biochimica et biophysica acta.

[119]  B. Ponder,et al.  Isolated familial pheochromocytoma as a variant of von Hippel-Lindau disease. , 1996, The Journal of clinical endocrinology and metabolism.

[120]  A. Webster,et al.  Phenotypic expression in von Hippel-Lindau disease: correlations with germline VHL gene mutations. , 1996, Journal of medical genetics.

[121]  R. Whitehouse,et al.  A genetic register for von Hippel-Lindau disease. , 1996, Journal of medical genetics.

[122]  P. Waber,et al.  Frequent allelic loss at chromosome arm 3p is distinct from genetic alterations of the Von-Hippel Lindau tumor suppressor gene in head and neck cancer. , 1996, Oncogene.

[123]  R. Conaway,et al.  Purification of RNA polymerase II general transcription factors from rat liver. , 1996, Methods in enzymology.

[124]  S. Peltz,et al.  Interrelationships of the pathways of mRNA decay and translation in eukaryotic cells. , 1996, Annual review of biochemistry.

[125]  W. Linehan,et al.  Germline mutations in the Von Hippel‐Lindau disease (VHL) gene in families from North America, Europe, and Japan , 1996, Human mutation.

[126]  B. Zbar,et al.  Familial pheochromocytoma associated with a novel mutation in the von Hippel-Lindau gene. , 1996, The Journal of clinical endocrinology and metabolism.

[127]  S. Chew,et al.  Mutations in the RET proto-oncogene and the von Hippel-Lindau disease tumour suppressor gene in sporadic and syndromic phaeochromocytomas. , 1995, Journal of medical genetics.

[128]  M. Ginsberg,et al.  Integrin activation and cytoskeletal interaction are essential for the assembly of a fibronectin matrix , 1995, Cell.

[129]  C. Bain,et al.  Somatic mutations of von Hippel‐Lindau (VHL) tumor‐suppressor gene in european kidney cancers , 1995, International journal of cancer.

[130]  Fan,et al.  Cellular proteins that bind the von Hippel-Lindau disease gene product: mapping of binding domains and the effect of missense mutations. , 1995, Cancer research.

[131]  A. Kibel,et al.  Binding of the von Hippel-Lindau tumor suppressor protein to Elongin B and C , 1995, Science.

[132]  William Arbuthnot Sir Lane,et al.  Elongin (SIII): a multisubunit regulator of elongation by RNA polymerase II , 1995, Science.

[133]  J. Nadol,et al.  Endolymphatic sac tumors: Histopathologic confirmation, clinical characterization, and implication in von hippel‐lindau disease , 1995, The Laryngoscope.

[134]  William Arbuthnot Sir Lane,et al.  Positive regulation of general transcription factor SIII by a tailed ubiquitin homolog. , 1995, Proceedings of the National Academy of Sciences of the United States of America.

[135]  A. Kibel,et al.  Tumour suppression by the human von Hippel-Lindau gene product , 1995, Nature Medicine.

[136]  R. Klausner,et al.  Characterization of the VHL tumor suppressor gene product: localization, complex formation, and the effect of natural inactivating mutations. , 1995, Proceedings of the National Academy of Sciences of the United States of America.

[137]  M. Goldberg,et al.  Transcriptional Regulation of the Rat Vascular Endothelial Growth Factor Gene by Hypoxia (*) , 1995, The Journal of Biological Chemistry.

[138]  J. Richie,et al.  Treatment of renal cell carcinoma in von Hippel-Lindau disease: a multicenter study. , 1995, The Journal of urology.

[139]  F. Latif,et al.  Identification of the promoter of the human von Hippel-Lindau disease tumor suppressor gene. , 1995, Oncogene.

[140]  F. Duh,et al.  Expression of the Von Hippel-Lindau Tumor Suppressor Gene, VHL, in Human Fetal Kidney and During Mouse Embryogenesis , 1995, Molecular Medicine.

[141]  Roy Parker,et al.  Degradation of mRNA in eukaryotes , 1995, Cell.

[142]  K. Plate,et al.  Up-regulation of vascular endothelial growth factor and its receptors in von Hippel-Lindau disease-associated and sporadic hemangioblastomas. , 1995, Cancer research.

[143]  P. Choyke,et al.  von Hippel-Lindau disease: genetic, clinical, and imaging features. , 1995, Radiology.

[144]  P. Choyke,et al.  Parenchymal sparing surgery in patients with hereditary renal cell carcinoma. , 1995, The Journal of urology.

[145]  B. Seizinger,et al.  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. , 1995, Cancer research.

[146]  G. Semenza,et al.  Purification and Characterization of Hypoxia-inducible Factor 1 (*) , 1995, The Journal of Biological Chemistry.

[147]  M. Kirschner,et al.  Ubiquitination of the G1 cyclin Cln2p by a Cdc34p‐dependent pathway. , 1995, The EMBO journal.

[148]  W. Linehan,et al.  Germline mutations in the von Hippel–Lindau disease tumor suppressor gene: Correlations with phenotype , 1995, Human mutation.

[149]  C. O'Brien,et al.  Supratentorial haemangioblastoma not associated with Von Hippel Lindau complex or polycythaemia: case report and literature review. , 1995, British journal of neurosurgery.

[150]  A. Gregor,et al.  Immunohistochemical and ultrastructural studies of stromal cells in hemangioblastoma. , 1995, Folia neuropathologica.

[151]  W. Sly,et al.  Human carbonic anhydrases and carbonic anhydrase deficiencies. , 1995, Annual review of biochemistry.

[152]  M. Ferguson-Smith,et al.  Somatic mutations of the von Hippel-Lindau disease tumour suppressor gene in non-familial clear cell renal carcinoma. , 1994, Human molecular genetics.

[153]  J. Nelson,et al.  The clinical and pathological manifestations of renal tumors in von Hippel-Lindau disease. , 1994, The Journal of urology.

[154]  B. Seizinger,et al.  Germ-line mutations in the von Hippel-Lindau tumor-suppressor gene are similar to somatic von Hippel-Lindau aberrations in sporadic renal cell carcinoma. , 1994, American journal of human genetics.

[155]  J. Herman,et al.  Silencing of the VHL tumor-suppressor gene by DNA methylation in renal carcinoma. , 1994, Proceedings of the National Academy of Sciences of the United States of America.

[156]  A. Bardelli,et al.  Identification of functional domains in the hepatocyte growth factor and its receptor by molecular engineering. , 1994, Journal of biotechnology.

[157]  T. Shuin,et al.  Somatic mutations of the von Hippel-Lindau tumor suppressor gene in sporadic central nervous system hemangioblastomas. , 1994, Cancer research.

[158]  R. Hené,et al.  Von Hippel-Lindau disease: new strategies in early detection and treatment. , 1994, The American journal of medicine.

[159]  M. Lerman,et al.  Identification of intragenic mutations in the von Hippel-Lindau disease tumour suppressor gene and correlation with disease phenotype. , 1994, Human molecular genetics.

[160]  T. Sugimura,et al.  Markedly increased amounts of messenger RNAs for vascular endothelial growth factor and placenta growth factor in renal cell carcinoma associated with angiogenesis. , 1994, Cancer research.

[161]  T. Sugiyama,et al.  Frequent overexpression of vascular endothelial growth factor gene in human renal cell carcinoma. , 1994, The Tohoku journal of experimental medicine.

[162]  William Arbuthnot Sir Lane,et al.  Molecular cloning of an essential subunit of RNA polymerase II elongation factor SIII. , 1994, Proceedings of the National Academy of Sciences of the United States of America.

[163]  J. Minna,et al.  Molecular analysis of the von Hippel-Lindau disease tumor suppressor gene in human lung cancer cell lines. , 1994, Oncogene.

[164]  Y Kubota,et al.  Frequent somatic mutations and loss of heterozygosity of the von Hippel-Lindau tumor suppressor gene in primary human renal cell carcinomas. , 1994, Cancer research.

[165]  D. Hough,et al.  Pancreatic lesions in von Hippel-Lindau disease: prevalence, clinical significance, and CT findings. , 1994, AJR. American journal of roentgenology.

[166]  J. Brooks,et al.  Mutations of the VHL tumour suppressor gene in renal carcinoma , 1994, Nature Genetics.

[167]  M. Lerman,et al.  Detailed mapping of germline deletions of the von Hippel-Lindau disease tumour suppressor gene. , 1994, Human molecular genetics.

[168]  M. Goldberg,et al.  Similarities between the oxygen-sensing mechanisms regulating the expression of vascular endothelial growth factor and erythropoietin. , 1994, The Journal of biological chemistry.

[169]  Wilson Pd,et al.  Renal progenitor cells: problems of definition, isolation and characterization. , 1994 .

[170]  S. Richard,et al.  Renal lesions and pheochromocytoma in von Hippel-Lindau disease. , 1994, Advances in nephrology from the Necker Hospital.

[171]  H. Neumann,et al.  Pheochromocytomas, multiple endocrine neoplasia type 2, and von Hippel-Lindau disease. , 1993, The New England journal of medicine.

[172]  R. Kuwano,et al.  Expression of vascular endothelial growth factor in capillary hemangioblastoma. , 1993, Biochemical and biophysical research communications.

[173]  J. Gnarra,et al.  Identification of the von Hippel-Lindau disease tumor suppressor gene. , 1993, Science.

[174]  M. Lush,et al.  Genetic linkage between von Hippel-Lindau disease and three microsatellite polymorphisms refines the localisation of the VHL locus. , 1993, Human molecular genetics.

[175]  M. Frydenberg,et al.  Conservative renal surgery for renal cell carcinoma in von Hippel-Lindau's disease. , 1993, The Journal of urology.

[176]  E Medico,et al.  Transfer of motogenic and invasive response to scatter factor/hepatocyte growth factor by transfection of human MET protooncogene. , 1993, Proceedings of the National Academy of Sciences of the United States of America.

[177]  G. H. Weiss,et al.  The natural history of renal lesions in von Hippel-Lindau disease: a serial CT study in 28 patients. , 1992, AJR. American journal of roentgenology.

[178]  H. Brambs,et al.  Pancreatic lesions in the von Hippel-Lindau syndrome. , 1991, Gastroenterology.

[179]  J. Caro,et al.  Secondary erythrocytosis due to a cerebellar hemangioblastoma: demonstration of erythropoietin mRNA in the tumor. , 1991, Blood.

[180]  J. Horton,et al.  Von Hippel‐Lindau disease and erythrocytosis: Radioimmunoassay of erythropoietin in cyst fluid from a brainstem hemangioblastoma , 1991, Neurology.

[181]  P. Choyke,et al.  Central nervous system involvement in Von Hippel‐Lindau disease , 1991, Neurology.

[182]  C. Scudamore,et al.  Hepatic hemangioblastoma. An unusual presentation in a patient with von Hippel-Lindau disease. , 1991, The American journal of surgical pathology.

[183]  M. Walther,et al.  Simple renal cysts, atypical renal cysts, and renal cell carcinoma in von Hippel-Lindau disease: a lectin and immunohistochemical study in six patients. , 1991, Modern Pathology.

[184]  M. Ferguson-Smith,et al.  Clinical features and natural history of von Hippel-Lindau disease. , 1990, The Quarterly journal of medicine.

[185]  C. Johnson,et al.  Islet cell tumors in von Hippel-Lindau disease: increased prevalence and relationship to the multiple endocrine neoplasias. , 1990, AJR. American journal of roentgenology.

[186]  R. Scully,et al.  Papillary cystadenoma of the broad ligament in von Hippel-Lindau disease. , 1990, American journal of obstetrics and gynecology.

[187]  Hsia Ye,et al.  von Hippel-Lindau disease affecting 43 members of a single kindred. , 1989 .

[188]  D. Solomon,et al.  Renal pathology in von Hippel-Lindau disease. , 1988, Human pathology.

[189]  J. Haines,et al.  Von Hippel–Lindau disease maps to the region of chromosome 3 associated with renal cell carcinoma , 1988, Nature.

[190]  H. Zincke,et al.  Renal cell carcinoma in von Hippel-Lindau syndrome. , 1987, The American journal of medicine.

[191]  J. Adamson,et al.  Polycythemia: mechanisms and management. , 1981, Annals of internal medicine.

[192]  K. Alitalo,et al.  FIBRONECTIN AND THE PERICELLULAR MATRIX OF NORMAL AND TRANSFORMED ADHERENT CELLS * , 1978, Annals of the New York Academy of Sciences.

[193]  K. Melmon,et al.  Lindau's disease: Review of the literature and study of a large kindred , 1964 .

[194]  A. Lindau.  ZUR FRAGE DER ANGIOMATOSIS RETINæ UND IHRER HIRNKOMPLIKATIONEN , 1926 .