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Updated: Jul 17, 2026

The Use of Reverse Phase Protein Arrays (RPPA) to Explore Protein Expression Variation within Individual Renal Cell Cancers
Published on: January 22, 2013
Identification of the genes for kidney cancer: opportunity for disease-specific targeted therapeutics
W Marston Linehan1, Peter A Pinto, Ramaprasad Srinivasan
1Urologic Oncology Branch, Center for Cancer Research, National Cancer Institute, Bethesda, MD 20892, USA. wml@nih.gov
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.
Insights
Understanding kidney cancer gene pathways, including von Hippel Lindau (VHL) and c-Met, aids targeted therapy development. Studying inherited forms reveals genetic defects for effective treatment strategies.
Area of Science:
- Oncology
- Genetics
- Molecular Biology
Background:
- Kidney cancer encompasses diverse subtypes with distinct characteristics and genetic underpinnings.
- Advances in understanding kidney cancer gene pathways are crucial for developing targeted therapies.
- Inherited kidney cancer syndromes (VHL, hereditary papillary renal carcinoma, BHD, hereditary leiomyomatosis renal cell cancer) illuminate the genetic basis of the disease.
Purpose of the Study:
- To explore the genetic basis of kidney cancer.
- To highlight the importance of inherited kidney cancer syndromes in understanding disease mechanisms.
- To emphasize the potential of targeted therapies based on identified gene pathways.
Main Methods:
- Review of current research on kidney cancer genetics.
- Analysis of inherited kidney cancer syndromes and their associated genes (VHL, c-Met, BHD, fumarate hydratase).
- Evaluation of targeted therapeutic strategies for specific kidney cancer pathways.
Main Results:
- Kidney cancer is a heterogeneous disease with diverse genetic causes.
- Inherited syndromes provide insights into key genes like VHL, c-Met, BHD, and fumarate hydratase.
- Targeting these specific pathways offers a promising therapeutic avenue.
Conclusions:
- Targeted therapies hold significant promise for kidney cancer treatment.
- Understanding the genetic landscape of kidney cancer is essential for personalized medicine.
- Further research into VHL, c-Met, BHD, and fumarate hydratase pathways can lead to improved patient outcomes.
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