Searching for the hereditary causes of renal-cell carcinoma

Distinct types of diffuse large B-cell lymphoma identified by gene expression profiling

2000 • 9,712 citations

Targeting of HIF-α to the von Hippel-Lindau Ubiquitylation Complex by O ₂ -Regulated Prolyl Hydroxylation

2001 • 5,255 citations

The tumour suppressor protein VHL targets hypoxia-inducible factors for oxygen-dependent proteolysis

1999 • 4,973 citations

HIFα Targeted for VHL-Mediated Destruction by Proline Hydroxylation: Implications for O ₂ Sensing

2001 • 4,537 citations

Identification of the von Hippel-Lindau Disease Tumor Suppressor Gene

1993 • 2,906 citations

A Randomized Trial of Bevacizumab, an Anti–Vascular Endothelial Growth Factor Antibody, for Metastatic Renal Cancer

2003 • 2,723 citations

Met, metastasis, motility and more

2003 • 2,526 citations

A Conserved Family of Prolyl-4-Hydroxylases That Modify HIF

2001 • 2,481 citations

Identification of the Hepatocyte Growth Factor Receptor As the c- met Proto-Oncogene Product

1991 • 2,230 citations

Molecular classification of cutaneous malignant melanoma by gene expression profiling

2000 • 2,011 citations

Regulation of Mammalian O₂Homeostasis by Hypoxia-Inducible Factor 1

1999 • 1,882 citations

Mutations of the VHL tumour suppressor gene in renal carcinoma

1994 • 1,719 citations

Rising Incidence of Renal Cell Cancer in the United States

1999 • 1,593 citations

Silencing of the VHL tumor-suppressor gene by DNA methylation in renal carcinoma.

1994 • 1,542 citations

Germline mutations in FH predispose to dominantly inherited uterine fibroids, skin leiomyomata and papillary renal cell cancer

2002 • 1,541 citations

Ubiquitination of hypoxia-inducible factor requires direct binding to the β-domain of the von Hippel–Lindau protein

2000 • 1,527 citations

Asparagine Hydroxylation of the HIF Transactivation Domain: A Hypoxic Switch

2002 • 1,512 citations

Germline and somatic mutations in the tyrosine kinase domain of the MET proto-oncogene in papillary renal carcinomas

1997 • 1,508 citations

von Hippel-Lindau disease

2003 • 1,496 citations

Frameshift Mutations and the Genetic Code

1966 • 1,400 citations

Scatter factor/hepatocyte growth factor is essential for liver development

1995 • 1,398 citations

The Heidelberg classification of renal cell tumours

1997 • 1,328 citations

FIH-1: a novel protein that interacts with HIF-1α and VHL to mediate repression of HIF-1 transcriptional activity

2001 • 1,326 citations

Essential role for the c-met receptor in the migration of myogenic precursor cells into the limb bud

1995 • 1,278 citations

Hypoxia promotes invasive growth by transcriptional activation of the met protooncogene

2003 • 1,276 citations

A mutation in the RET proto-oncogene associated with multiple endocrine neoplasia type 2B and sporadic medullary thyroid carcinoma

1994 • 1,140 citations

Placental defect and embryonic lethality in mice lacking hepatocyte growth factor/scatter factor

1995 • 1,039 citations

Hypoxia Inducible Factor-α Binding and Ubiquitylation by the von Hippel-Lindau Tumor Suppressor Protein

2000 • 1,018 citations

The FHIT Gene, Spanning the Chromosome 3p14.2 Fragile Site and Renal Carcinoma–Associated t(3;8) Breakpoint, Is Abnormal in Digestive Tract Cancers

1996 • 1,008 citations

Identification of a point mutation in the catalytic domain of the protooncogene c-kit in peripheral blood mononuclear cells of patients who have mastocytosis with an associated hematologic disorder.

1995 • 895 citations

Negative regulation of hypoxia-inducible genes by the von Hippel-Lindau protein.

1996 • 886 citations

Mutations in a novel gene lead to kidney tumors, lung wall defects, and benign tumors of the hair follicle in patients with the Birt-Hogg-Dubé syndrome

2002 • 882 citations

Chemokine receptor CXCR4 downregulated by von Hippel–Lindau tumour suppressor pVHL

2003 • 864 citations

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

1999 • 834 citations

Molecular basis of the VHL hereditary cancer syndrome

2002 • 826 citations

Inhibition of HIF is necessary for tumor suppression by the von Hippel-Lindau protein

2002 • 754 citations

Scatter-factor and semaphorin receptors: cell signalling for invasive growth

2002 • 734 citations

HRPT2, encoding parafibromin, is mutated in hyperparathyroidism–jaw tumor syndrome

2002 • 729 citations

Hereditary Multiple Fibrofolliculomas With Trichodiscomas and Acrochordons

1977 • 711 citations

Structure of an HIF-1α-pVHL Complex: Hydroxyproline Recognition in Signaling

2002 • 707 citations

Tumour suppression by the human von Hippel-Lindau gene product

1995 • 705 citations

Structural basis for the recognition of hydroxyproline in HIF-1α by pVHL

2002 • 689 citations

Hereditary Renal-Cell Carcinoma Associated with a Chromosomal Translocation

1979 • 649 citations

Inherited susceptibility to uterine leiomyomas and renal cell cancer

2001 • 641 citations

Mutations in the Fumarate Hydratase Gene Cause Hereditary Leiomyomatosis and Renal Cell Cancer in Families in North America

2003 • 605 citations

Inhibition of HIF2α Is Sufficient to Suppress pVHL-Defective Tumor Growth

2003 • 579 citations

Renal Tumors in the Birt-Hogg-Dubé Syndrome

2002 • 578 citations

Somatic and Germ-Line Mutations of theHRPT2Gene in Sporadic Parathyroid Carcinoma

2003 • 574 citations

Germline mutations in the von Hippel-Lindau disease tumor suppressor gene: Correlations with phenotype

1995 • 552 citations

Post-transcriptional regulation of vascular endothelial growth factor mRNA by the product of the VHL tumor suppressor gene.

1996 • 541 citations

von Hippel-Lindau Disease

1997 • 506 citations

Novel mutations of the MET proto-oncogene in papillary renal carcinomas

1999 • 502 citations

Germline mutations in the Von Hippel-Lindau disease (VHL) gene in families from North America, Europe, and Japan

1996 • 490 citations

The von Hippel-Lindau tumor-suppressor gene product forms a stable complex with human CUL-2, a member of the Cdc53 family of proteins

1997 • 490 citations

Risk of renal and colonic neoplasms and spontaneous pneumothorax in the Birt-Hogg-Dubé syndrome.

2002 • 481 citations

The von Hippel-Lindau Tumor Suppressor Protein Is Required for Proper Assembly of an Extracellular Fibronectin Matrix

1998 • 474 citations

HIF activation identifies early lesions in VHL kidneys

2002 • 469 citations

Frequent somatic mutations and loss of heterozygosity of the von Hippel-Lindau tumor suppressor gene in primary human renal cell carcinomas.

1994 • 469 citations

Improved detection of germline mutations in the von Hippel-Lindau disease tumor suppressor gene

1998 • 465 citations

Activating mutations for the Met tyrosine kinase receptor in human cancer

1997 • 441 citations

The contribution of VHL substrate binding and HIF1-α to the phenotype of VHL loss in renal cell carcinoma

2002 • 438 citations

Gene expression profiling of clear cell renal cell carcinoma: Gene identification and prognostic classification

2001 • 408 citations

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

2001 • 402 citations

A role for mitochondrial enzymes in inherited neoplasia and beyond

2003 • 387 citations

Early-Onset Renal Cell Carcinoma as a Novel Extraparaganglial Component of SDHB-Associated Heritable Paraganglioma

2004 • 380 citations

von Hippel-Lindau protein mutants linked to type 2C VHL disease preserve the ability to downregulate HIF

2001 • 374 citations

Somatic mutations of the von Hippel — Lindau disease tumour suppressor gene in non-familial clear cell renal carcinoma

1994 • 371 citations

Replacement of Fhit in cancer cells suppresses tumorigenicity

1997 • 368 citations

Birt-Hogg-Dubé Syndrome

1999 • 366 citations

Morphologic typing of papillary renal cell carcinoma: Comparison of growth kinetics and patient survival in 66 cases

2001 • 366 citations

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

2001 • 364 citations

HRPT2 mutations are associated with malignancy in sporadic parathyroid tumours

2003 • 364 citations

Birt-Hogg-Dubé Syndrome, a Genodermatosis Associated with Spontaneous Pneumothorax and Kidney Neoplasia, Maps to Chromosome 17p11.2

2001 • 360 citations

Defective placental vasculogenesis causes embryonic lethality in VHL-deficient mice

1997 • 359 citations

Regulation of microtubule stability by the von Hippel-Lindau tumour suppressor protein pVHL

2002 • 330 citations

PERCUTANEOUS RADIO FREQUENCY ABLATION OF SMALL RENAL TUMORS: INITIAL RESULTS

2002 • 317 citations

Reversion of deregulated expression of vascular endothelial growth factor in human renal carcinoma cells by von Hippel-Lindau tumor suppressor protein.

1996 • 315 citations

GENETIC AND EPIGENETIC ALTERATIONS IN COLON CANCER

2002 • 313 citations

Genetic and functional analyses of FH mutations in multiple cutaneous and uterine leiomyomatosis, hereditary leiomyomatosis and renal cancer, and fumarate hydratase deficiency

2003 • 304 citations

Trisomy 7-harbouring non-random duplication of the mutant MET allele in hereditary papillary renal carcinomas

1998 • 285 citations

Gene Expression Patterns in Renal Cell Carcinoma Assessed by Complementary DNA Microarray

2003 • 262 citations

Hereditary hyperparathyroidism-jaw tumor syndrome: the endocrine tumor gene HRPT2 maps to chromosome 1q21-q31.

1995 • 247 citations

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

1998 • 244 citations

A novel small molecule met inhibitor induces apoptosis in cells transformed by the oncogenic TPR-MET tyrosine kinase.

2003 • 237 citations

Chromosomal Imbalances in Papillary Renal Cell Carcinoma

1998 • 232 citations

The mutationally activated Met receptor mediates motility and metastasis

1998 • 228 citations

Hypoxia-inducible Protein Binding to Vascular Endothelial Growth Factor mRNA and Its Modulation by the von Hippel-Lindau Protein

1996 • 212 citations

Clinical and genetic studies of Birt-Hogg-Dube syndrome

2002 • 212 citations

PARENCHYMAL SPARING SURGERY IN PATIENTS WITH HEREDITARY RENAL CELL CARCINOMA: 10-YEAR EXPERIENCE

2001 • 211 citations

Familial Cutaneous Leiomyomatosis Is a Two-Hit Condition Associated with Renal Cell Cancer of Characteristic Histopathology

2001 • 204 citations

Renal Oncocytosis

1999 • 203 citations

Hereditary hyperparathyroidism and multiple ossifying jaw fibromas: a clinically and genetically distinct syndrome.

1990 • 202 citations

The von Hippel–Lindau tumor suppressor, hypoxia-inducible factor-1 (HIF-1) degradation, and cancer pathogenesis

2002 • 202 citations

Birt-Hogg-Dubé syndrome: mapping of a novel hereditary neoplasia gene to chromosome 17p12-q11.2

2001 • 201 citations

The von Hippel-Lindau Tumor Suppressor Gene Inhibits Hepatocyte Growth Factor/Scatter Factor-Induced Invasion and Branching Morphogenesis in Renal Carcinoma Cells

1999 • 200 citations

Papillary Thyroid Carcinoma Associated with Papillary Renal Neoplasia: Genetic Linkage Analysis of a Distinct Heritable Tumor Syndrome*

2000 • 194 citations

Allelic deletions of the VHL gene detected in multiple microscopic clear cell renal lesions in von Hippel-Lindau disease patients.

1996 • 187 citations

A mutation in the canine BHD gene is associated with hereditary multifocal renal cystadenocarcinoma and nodular dermatofibrosis in the German Shepherd dog

2003 • 185 citations

Two North American Families With Hereditary Papillary Renal Carcinoma and Identical Novel Mutations in the MET Proto-Oncogene

1999 • 184 citations

Role of transforming growth factor-alpha in von Hippel--Lindau (VHL)(-/-) clear cell renal carcinoma cell proliferation: a possible mechanism coupling VHL tumor suppressor inactivation and tumorigenesis.

2001 • 184 citations

Predicting survival in patients with metastatic kidney cancer by gene-expression profiling in the primary tumor

2003 • 179 citations

The von Hippel–Lindau tumor suppressor protein: new insights into oxygen sensing and cancer

2003 • 172 citations

Treatment of Renal Cell Carcinoma in Von Hippel-Lindau Disease: A Multicenter Study

1995 • 172 citations

Transforming growth factor alpha is a target for the von Hippel-Lindau tumor suppressor.

1998 • 170 citations

The geldanamycins are potent inhibitors of the hepatocyte growth factor/scatter factor-met-urokinase plasminogen activator-plasmin proteolytic network.

2000 • 167 citations

Recent advances in Wilms tumor genetics

2002 • 164 citations

Original Articles: Kidney Cancer: Hereditary Papillary Renal Cell Carcinoma: Clinical Studies in 10 Families

1995 • 163 citations

Few FH mutations in sporadic counterparts of tumor types observed in hereditary leiomyomatosis and renal cell cancer families.

2002 • 159 citations

Localization of a Gene (MCUL1) for Multiple Cutaneous Leiomyomata and Uterine Fibroids to Chromosome 1q42.3-q43

2001 • 158 citations

Autosomal dominant primary hyperparathyroidism and jaw tumor syndrome associated with renal hamartomas and cystic kidney disease: linkage to 1q21-q32 and loss of the wild type allele in renal hamartomas.

1996 • 158 citations

The hereditary renal cell carcinoma 3;8 translocation fuses FHIT to a patched- related gene, TRC8

1998 • 154 citations

Molecular genetic investigations of the mechanism of tumourigenesis in von Hippel-Lindau disease: analysis of allele loss in VHL tumours

1994 • 150 citations

Genotype-phenotype correlation in von Hippel-Lindau syndrome

2001 • 148 citations

The Tuberous Sclerosis Complex and its Highly Variable Manifestations

2003 • 146 citations

Tissue‐specific expression of a constitutional 3;6 translocation: Development of multiple bilateral renal‐cell carcinomas

1989 • 142 citations

Inactivation of BHD in sporadic renal tumors.

2003 • 141 citations

Mutant Met-mediated transformation is ligand-dependent and can be inhibited by HGF antagonists

1999 • 138 citations

Duplication and overexpression of the mutant allele of the MET proto-oncogene in multiple hereditary papillary renal cell tumours

1998 • 136 citations

FAMILIAL RENAL ONCOCYTOMA: CLINICOPATHOLOGICAL STUDY OF 5 FAMILIES

1998 • 134 citations

HIF-1

2003 • 132 citations

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

2003 • 130 citations

Tumour suppressors hamartin and tuberin: intracellular signalling

2003 • 127 citations

VHL-mediated hypoxia regulation of cyclin D1 in renal carcinoma cells.

2002 • 123 citations

The t(1;3) breakpoint-spanning genes LSAMP and NORE1 are involved in clear cell renal cell carcinomas

2003 • 118 citations

PERCUTANEOUS RENAL CRYOABLATION OF RENAL TUMORS IN PATIENTS WITH VON HIPPEL-LINDAU DISEASE

2002 • 113 citations

Hepatic vascular tumors, angiectasis in multiple organs, and impaired spermatogenesis in mice with conditional inactivation of the VHL gene.

2003 • 113 citations

Transgenic expression of tpr-met oncogene leads to development of mammary hyperplasia and tumors.

1996 • 112 citations

A Genotypic and Histopathological Study of a Large Dutch Kindred with Hyperparathyroidism-Jaw Tumor Syndrome1

2000 • 109 citations

Suppression of growth of renal carcinoma cells by the von Hippel-Lindau tumor suppressor gene.

1995 • 107 citations

Tumorigenic Mutations in VHL Disrupt Folding In Vivo by Interfering with Chaperonin Binding

2003 • 106 citations

VHL Induces Renal Cell Differentiation and Growth Arrest through Integration of Cell-Cell and Cell-Extracellular Matrix Signaling

2001 • 105 citations

Loss of von Hippel-Lindau protein causes cell density dependent deregulation of CyclinD1 expression through Hypoxia-inducible factor

2003 • 104 citations

Hereditary Multifocal Renal Cystadenocarcinomas and Nodular Dermatofibrosis in the German Shepherd Dog: Macroscopic and Histopathologic Changes

1985 • 101 citations

Genetic mapping of a naturally occurring hereditary renal cancer syndrome in dogs

2000 • 101 citations

Understanding familial and non-familial renal cell cancer

2002 • 97 citations

Solid renal tumor severity in von Hippel Lindau disease is related to germline deletion length and location

2003 • 96 citations

A germ-line insertion in the Birt–Hogg–Dubé ( BHD ) gene gives rise to the Nihon rat model of inherited renal cancer

2004 • 96 citations

Pathway specificity for Met signalling

2001 • 95 citations

Novel mutation in the ATP-binding site of theMET oncogene tyrosine kinase in a HPRCC family

1999 • 94 citations

A population‐based familial aggregation analysis indicates genetic contribution in a majority of renal cell carcinomas

2002 • 90 citations

Molecular Analysis and Prenatal Diagnosis of Human Fumarase Deficiency

1998 • 78 citations

An Alternative Route for Multistep Tumorigenesis in a Novel Case of Hereditary Renal Cell Cancer and a t(2;3)(q35;q21) Chromosome Translocation

1998 • 74 citations

Analysis of the Birt-Hogg-Dube (BHD) tumour suppressor gene in sporadic renal cell carcinoma and colorectal cancer

2003 • 70 citations

Low frequency of somatic mutations in the FH/multiple cutaneous leiomyomatosis gene in sporadic leiomyosarcomas and uterine leiomyomas

2002 • 69 citations

Studying Cancer Families to Identify Kidney Cancer Genes

2003 • 67 citations

SU5416 plus interferon alpha in advanced renal cell carcinoma: a phase II California Cancer Consortium Study with biological and imaging correlates of angiogenesis inhibition.

2003 • 67 citations

Family history and risk of renal cell carcinoma.

2001 • 66 citations

Structural basis of oncogenic activation caused by point mutations in the kinase domain of the MET proto‐oncogene: Modeling studies

2001 • 64 citations

The TRC8 hereditary kidney cancer gene suppresses growth and functions with VHL in a common pathway

2002 • 63 citations

Familial clear cell renal cell carcinoma (FCRC): clinical features and mutation analysis of the VHL, MET, and CUL2 candidate genes

2000 • 54 citations

Mutations in the met Oncogene Unveil a “Dual Switch” Mechanism Controlling Tyrosine Kinase Activity

2003 • 53 citations

Tissue-specific transformation by epidermal growth factor receptor: a single point mutation within the ATP-binding pocket of the erbB product increases its intrinsic kinase activity and activates its sarcomagenic potential.

1990 • 50 citations

Disruption of a novel gene, DIRC3, and expression of DIRC3‐HSPBAP1 fusion transcripts in a case of familial renal cell cancer and t(2;3)(q35;q21)

2003 • 49 citations

Disruption of a novel MFS transporter gene, DIRC2, by a familial renal cell carcinoma-associated t(2;3)(q35;q21)

2002 • 47 citations

Thalidomide therapy for renal cell carcinoma

2003 • 47 citations

Low frequency of somatic mutations in the FH/multiple cutaneous leiomyomatosis gene in sporadic leiomyosarcomas and uterine leiomyomas

2002 • 46 citations

Somatic point mutation of the wild-type allele detected in tumors of patients with VHL germline deletion

2002 • 43 citations

Mechanism of tumorigenesis of renal carcinomas associated with the constitutional chromosome 3;8 translocation.

1995 • 42 citations

A Novel “Nihon'’Rat Model of a Mendelian Dominantly Inherited Renal Cell Carcinoma

2000 • 35 citations

Imaging of hereditary renal cancer

2003 • 35 citations

Precise localization of theFHIT gene to the common fragile site at 3p14.2 (FRA3B) and characterization of homozygous deletions within FRA3B that affectFHIT transcription in tumor cell lines

1997 • 29 citations

A Novel Renal Carcinoma Predisposing Gene of the Nihon Rat Maps on Chromosome 10

2001 • 27 citations

The DIRC1 gene at chromosome 2q33 spans a familial RCC-associated t(2;3)(q33;q21) chromosome translocation

2001 • 25 citations

Germline mutations in fumarate hydratase (FH) do not predispose to prostate cancer

2003 • 9 citations