Prions and cancer: A story unfolding

June 25, 2012

Prions, the causal agents of Mad Cow and other diseases, are very unique infectious particles. They are proteins in which the complex molecular three-dimensional folding process just went astray. For reasons not yet understood, the misfolding nature of prions is associated to their ability to sequester their normal counterparts and induce them to also adopt a misfolding conformation. The ever-growing crowd of misfolded proteins form the aggregates seen in diseases such as Parkinson's and Alzheimer's. Once misfolded, a protein can no longer exert its normal functions in the cell.

Now, a group led by Dr Jerson Lima Silva at the Federal University of Rio de Janeiro, Brazil, presents some new evidence that p53, a protein with the daunting task of suppressing tumor formation in the body, may show a typical prion-like behavior when mutated.

It has been known for some time that the buildup of p53 in the cell impairs the protein in preventing tumor growth. This has been observed in neuroblastoma, retinoblastoma, breast, and colon cancers. In a paper entitled "Mutant p53 aggregates into prion-like amyloid oligomers and fibrils: Implications for cancer" and published in the Journal of Biological Chemistry, the group shows that in cell lines carrying the most common , the formation of amyloid-like aggregates of p53 proteins may explain the protein's lack of function.

Whether this prionoid behavior in fact represents a relevant cancer-related mechanism remains to be shown. Development of novel and ingenious strategies to prevent p53 misfolding and aggregation may be just one way to find out.

"We are planning pre-clinical tests with synthesized in an attempt to prevent the changing in conformation of normal p53, and avoid aggregates of misfolded protein," says Dr. Silva.

If successful, the strategy may help unveil unforeseen leading to tumor development. Considering that more than half of the cancers lose p53 function, this prionoid behavior may serve as a potential novel target for cancer therapy, dramatically transforming our way of thinking of cancer and treating cancer patients.

Explore further: Drug kills cancer cells by restoring faulty tumor suppressor

More information: Journal of Biological Chemistry doi: 10.1074/jbc.M112.340638

Related Stories

Drug kills cancer cells by restoring faulty tumor suppressor

May 14, 2012
A new study describes a compound that selectively kills cancer cells by restoring the structure and function of one of the most commonly mutated proteins in human cancer, the "tumor suppressor" p53. The research, published ...

Cellular stress can induce yeast to promote prion formation

July 23, 2011
It's a chicken and egg question. Where do the infectious protein particles called prions come from? Essentially clumps of misfolded proteins, prions cause neurodegenerative disorders, such as mad cow/Creutzfeld-Jakob disease, ...

Recommended for you

How cancer metastasis happens: Researchers reveal a key mechanism

January 18, 2018
Cancer metastasis, the migration of cells from a primary tumor to form distant tumors in the body, can be triggered by a chronic leakage of DNA within tumor cells, according to a team led by Weill Cornell Medicine and Memorial ...

Modular gene enhancer promotes leukemia and regulates effectiveness of chemotherapy

January 18, 2018
Every day, billions of new blood cells are generated in the bone marrow. The gene Myc is known to play an important role in this process, and is also known to play a role in cancer. Scientists from the German Cancer Research ...

These foods may up your odds for colon cancer

January 18, 2018
(HealthDay)—Chowing down on red meat, white bread and sugar-laden drinks might increase your long-term risk of colon cancer, a new study suggests.

The pill lowers ovarian cancer risk, even for smokers

January 18, 2018
(HealthDay)—It's known that use of the birth control pill is tied to lower odds for ovarian cancer, but new research shows the benefit extends to smokers or women who are obese.

Researchers develop swallowable test to detect pre-cancerous Barrett's esophagus

January 17, 2018
Investigators at Case Western Reserve University School of Medicine and University Hospitals Cleveland Medical Center have developed a simple, swallowable test for early detection of Barrett's esophagus that offers promise ...

Scientists zoom in to watch DNA code being read

January 17, 2018
Scientists have unveiled incredible images of how the DNA code is read and interpreted—revealing new detail about one of the fundamental processes of life.

0 comments

Please sign in to add a comment. Registration is free, and takes less than a minute. Read more

Click here to reset your password.
Sign in to get notified via email when new comments are made.