title: Identification of the genes for kidney cancer: opportunity for disease-specific targeted therapeutics.
abstract: Recent advances in understanding the kidney cancer gene pathways has provided the foundation for the development of targeted therapeutic approaches for patients with this disease. Kidney cancer is not a single disease; it includes a number of different types of renal cancers, each with different histologic features, a different clinical course, a different response to therapy, and different genes causing the defects. Most of what is known about the genetic basis of kidney cancer has been learned from study of the inherited forms of kidney cancer: von Hippel Lindau (VHL gene), hereditary papillary renal carcinoma (c-Met gene), Birt Hogg Dubé (BHD gene), and hereditary leiomyomatosis renal cell cancer (fumarate hydratase gene). These Mendelian single-gene syndromes provide a unique opportunity to evaluate the effectiveness of agents that target the VHL, c-Met, BHD, and fumarate hydratase pathways.
authors.affiliation: Urologic Oncology Branch, Center for Cancer Research, National Cancer Institute, Bethesda, MD 20892, USA. wml@nih.gov
authors.forename: W Marston
authors.initials: WM
authors.lastname: Linehan
authors.forename: Peter A
authors.initials: PA
authors.lastname: Pinto
authors.forename: Ramaprasad
authors.initials: R
authors.lastname: Srinivasan
authors.forename: Maria
authors.initials: M
authors.lastname: Merino
authors.forename: Peter
authors.initials: P
authors.lastname: Choyke
authors.forename: Lynda
authors.initials: L
authors.lastname: Choyke
authors.forename: Jonathan
authors.initials: J
authors.lastname: Coleman
authors.forename: Jorge
authors.initials: J
authors.lastname: Toro
authors.forename: Gladys
authors.initials: G
authors.lastname: Glenn
authors.forename: Cathy
authors.initials: C
authors.lastname: Vocke
authors.forename: Bert
authors.initials: B
authors.lastname: Zbar
authors.forename: Laura S
authors.initials: LS
authors.lastname: Schmidt
authors.forename: Donald
authors.initials: D
authors.lastname: Bottaro
authors.forename: Len
authors.initials: L
authors.lastname: Neckers
mesh_terms: D005260:Female; D020022:Genetic Predisposition to Disease; D006801:Humans; D007680:Kidney Neoplasms / Q000175:diagnosis / Q000188:drug therapy* / Q000235:genetics*; D008297:Male; D008954:Models, Biological; D008957:Models, Genetic; D009154:Mutation; D010597:Pharmacogenetics; D025521:Tumor Suppressor Proteins / Q000378:metabolism; D051794:Von Hippel-Lindau Tumor Suppressor Protein / Q000235:genetics
chemical_list: D025521:Tumor Suppressor Proteins; D051794:Von Hippel-Lindau Tumor Suppressor Protein; C497446:VHL protein, human