Related Experiment Video

Updated: Jul 18, 2026

Comparing Metastatic Clear Cell Renal Cell Carcinoma Model Established in Mouse Kidney and on Chicken Chorioallantoic Membrane
05:36

Comparing Metastatic Clear Cell Renal Cell Carcinoma Model Established in Mouse Kidney and on Chicken Chorioallantoic Membrane

Published on: February 8, 2020

Targeting von Hippel-Lindau pathway in renal cell carcinoma

Premal H Patel1, Rajendrakumar S V Chadalavada, R S K Chaganti

  • 1Department of Medicine, Memorial Sloan-Kettering Cancer Center, New York, New York 10021, USA.

Clinical Cancer Research : an Official Journal of the American Association for Cancer Research
|December 26, 2006
PubMed

Insights

Defective von Hippel-Lindau (VHL) gene causes inherited kidney cancer (RCC). Targeting the VHL/hypoxia pathway with novel agents significantly improves progression-free survival for RCC patients.

Area of Science:

  • Oncology
  • Genetics
  • Molecular Biology

Background:

  • The von Hippel-Lindau (VHL) gene is crucial in preventing kidney cancer (RCC).
  • Defects in VHL are linked to both inherited and sporadic forms of RCC.
  • VHL gene absence causes hypoxia-inducible factor alpha accumulation, promoting tumor growth via growth factors.

Purpose of the Study:

  • To review the biology of renal cell carcinoma (RCC).
  • To explore the therapeutic potential of targeting the VHL/hypoxia-inducible factor alpha pathway.
  • To assess the impact of novel targeted agents on RCC progression.

Main Methods:

  • Review of existing literature on VHL gene biology and RCC.
  • Analysis of targeted agents affecting the VHL/hypoxia-inducible factor alpha pathway.
  • Evaluation of clinical outcomes, focusing on progression-free survival.

Main Results:

  • VHL gene defects are a primary cause of inherited RCC and common in sporadic RCC.
  • Accumulation of hypoxia-inducible factor alpha, due to VHL absence, drives RCC pathogenesis.
  • Targeted agents like sunitinib, sorafenib, bevacizumab, everolimus, and temsirolimus show promise.

Conclusions:

  • Blocking the VHL/hypoxia-inducible factor alpha pathway offers a viable therapeutic strategy for RCC.
  • Novel targeted agents have demonstrated significant improvements in progression-free survival for RCC patients.
  • Further research into combination therapies targeting this pathway is warranted.

More Related Videos

Modeling Spontaneous Metastatic Renal Cell Carcinoma (mRCC) in Mice Following Nephrectomy11:27

Modeling Spontaneous Metastatic Renal Cell Carcinoma (mRCC) in Mice Following Nephrectomy

Published on: April 29, 2014

Related Concept Videos

The Retinoblastoma Gene01:20

The Retinoblastoma Gene

Tumor suppressor genes are normal genes that can slow down cell division, repair DNA mistakes, or program the cells for apoptosis in case of irreparable damage. Hence, they play an essential role in preventing the proliferation of damaged cells.
The first-ever tumor suppressor gene called Rb was identified in retinoblastoma - a rare eye tumor in children. In inherited forms of the disease, a child inherits one defective copy of the Rb gene, which predisposes them to retinoblastoma. However,...
Targeted Cancer Therapies02:57

Targeted Cancer Therapies

The targeted cancer therapies, also known as “molecular targeted therapies,” take advantage of the molecular and genetic differences between the cancer cells and the normal cells. It needs a thorough understanding of the cancer cells to develop drugs that can target specific molecular aspects that drive the growth, progression, and spread of cancer cells without affecting the growth and survival of other normal cells in the body.
There are several types of targeted therapies against specific...
Regulation of Angiogenesis and Blood Supply01:24

Regulation of Angiogenesis and Blood Supply

Rapidly dividing tumors, embryos, and wounded tissues require more oxygen than usual, lowering the oxygen concentration in the blood. At low oxygen or hypoxic conditions, an oxygen-sensitive transcription factor called the hypoxia-inducible factor 1 or HIF1 is activated. HIF1 is a dimeric protein of alpha (ɑ) and beta (β) subunits.  Under optimal oxygen conditions, HIF1β is present in the nucleus while HIF1ɑ remains in the cytosol. HIF1ɑ is hydroxylated by prolyl hydroxylase and factor...
mTOR Signaling and Cancer Progression03:03

mTOR Signaling and Cancer Progression

The mammalian target of rapamycin or mTOR protein was discovered in 1994 due to its direct interaction with rapamycin. The protein gets its name from a yeast homolog called TOR. The mTOR protein complex in mammalian cells plays a major role in balancing anabolic processes such as the synthesis of proteins, lipids, and nucleotides and catabolic processes, such as autophagy in response to environmental cues, such as availability of nutrients and growth factors.
The mTOR pathway or the...
Inhibition of Cdk Activity02:34

Inhibition of Cdk Activity

The orderly progression of the cell cycle depends on the activation of Cdk protein by binding to its cyclin partner. However, the cell cycle must be restricted when undergoing abnormal changes. Most cancers correlate to the deregulated cell cycle, and since Cdks are a central component of the cell cycle, Cdk inhibitors are extensively studied to develop anticancer agents. For instance, cyclin D associates with several Cdks, such as Cdk 4/6, to form an active complex. The cyclin D-Cdk4/6 complex...
M-Cdk Drives Transition Into Mitosis02:15

M-Cdk Drives Transition Into Mitosis

Checkpoints throughout the cell cycle serve as safeguards and gatekeepers, allowing the cell cycle to progress in favorable conditions and slow or halt it in problematic ones. This regulation is known as the cell cycle control system.
Cyclin-dependent kinases, or Cdks, work in concert with cyclins to control cell cycle transitions. M-Cdk, a complex of Cdk1 bound to M cyclin, is a well-known example of this coordinated control that drives the transition from the G2 to the M phase.
M cyclin...