Related Experiment Video

Updated: Jul 2, 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

[Von-Hippel-Lindau (VHL) protein function by initiation and progression of renal cancer]

H Moch1

  • 1Institut für Klinische Pathologie, Departement Pathologie, Universitätsspital Zürich, Schmelzbergstr. 12, 8091 Zürich, Schweiz. holger.moch@usz.ch

Der Pathologe
|August 30, 2008
PubMed

Insights

The von Hippel-Lindau (VHL) tumor suppressor gene and its protein pVHL are crucial for preventing cancers. VHL gene mutations lead to VHL syndrome and various tumors by affecting oxygen sensing and other cell processes.

Area of Science:

  • Oncology
  • Molecular Biology
  • Genetics

Background:

  • Germline inactivation of the VHL tumor suppressor gene causes von Hippel-Lindau hereditary cancer syndrome.
  • Somatic mutations in the VHL gene are associated with sporadic hemangioblastomas and clear cell renal carcinomas.
  • The VHL protein (pVHL) functions as an adaptor protein without enzymatic activity, interacting with multiple partners.

Purpose of the Study:

  • To review and highlight the diverse functions of the pVHL protein.
  • To elucidate the role of pVHL in gene expression regulation and oxygen sensing.
  • To explore recently identified HIFalpha-independent functions of pVHL.

Main Methods:

  • Literature review of VHL gene and pVHL protein functions.
  • Analysis of pVHL's role in oxygen-dependent regulation of hypoxia-inducible factor alpha (HIFalpha).
  • Examination of HIFalpha-independent cellular processes regulated by pVHL.

Main Results:

  • pVHL acts as a multi-purpose adaptor protein controlling gene expression programs.
  • pVHL is central to the oxygen-sensing pathway via HIFalpha regulation.
  • Identified HIFalpha-independent functions include microtubule processes, extracellular matrix assembly, and kidney cyst suppression.

Conclusions:

  • Complex pVHL functions explain its role in tumor formation and progression.
  • Dysregulation of pVHL contributes to diverse oncogenic pathways.
  • Understanding pVHL's multifaceted roles is key to VHL-related disease research.

More Related Videos

A Comprehensive Procedure to Evaluate the In Vitro Performance of the Putative Hemangioblastoma Neovascularization Using the Spheroid Sprouting Assay08:26

A Comprehensive Procedure to Evaluate the In Vitro Performance of the Putative Hemangioblastoma Neovascularization Using the Spheroid Sprouting Assay

Published on: April 12, 2018

Related Concept Videos

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...
Tumor Progression02:07

Tumor Progression

Tumor progression is a phenomenon where the pre-formed tumor acquires successive mutations to become clinically more aggressive and malignant. In the 1950s, Foulds first described the stepwise progression of cancer cells through successive stages.
Colon cancer is one of the best-documented examples of tumor progression. Early mutation in the APC gene in colon cells causes a small growth on the colon wall called a polyp. With time, this polyp grows into a benign, pre-cancerous tumor. Further...
Tumor Progression02:07

Tumor Progression

Tumor progression is a phenomenon where the pre-formed tumor acquires successive mutations to become clinically more aggressive and malignant. In the 1950s, Foulds first described the stepwise progression of cancer cells through successive stages.
Colon cancer is one of the best-documented examples of tumor progression. Early mutation in the APC gene in colon cells causes a small growth on the colon wall called a polyp. With time, this polyp grows into a benign, pre-cancerous tumor. Further...
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,...
Hedgehog Signaling Pathway02:33

Hedgehog Signaling Pathway

The Hedgehog gene (Hh) was first discovered due to its control of the growth of disorganized, hair-like bristles phenotype in Drosophila, much like hedgehog spines. Hh plays a crucial role in the development of organs and the maintenance of homeostasis in both invertebrates and vertebrates. However, while Drosophila has only one Hh protein, mammals have multiple functional Hedgehog proteins - Sonic (Shh), Desert (Dhh), and Indian Hedgehog (Ihh). All of these homologous proteins have adapted to...
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...