Research identifies genes vital to preventing childhood leukemia

July 18, 2011

Researchers at The University of Western Ontario have identified genes that may be important for preventing childhood leukemia. Acute lymphoblastic leukemia (ALL) is a cancer of the blood that occurs primarily in young children. It's frequently associated with mutations or chromosomal abnormalities that arise during embryonic or fetal development. Working with mice, researchers led by Rodney DeKoter identified two key genes that appear essential in the prevention of B cell ALL, the most common form of ALL in children. The study is published online in Blood, the Journal of the American Society of Hematology.

In the study, mice were generated with mutations in two genes called PU.1 and Spi-B. Mutation of either PU.1 or Spi-B individually had little effect. Unexpectedly, mutation of both genes resulted in 100% of the mice developing ALL. Eighty percent of ALL cases in children are of the B cell type. The study found PU.1 and Spi-B have unanticipated functional redundancy as "tumor suppressor" genes that prevent leukemia.

"You can think of PU.1 and Spi-B proteins as brakes on a car. If the main brake (PU.1) fails, you still have the emergency brake (Spi-B). However, if both sets of brakes fail, the car speeds out of control," explains DeKoter, an associate professor in the Department of Microbiology & Immunology at Western's Schulich School of Medicine & Dentistry. "And uncontrolled cell division is an important cause of leukemia."

PU.1 is an essential regulator in the development of the immune system, and mutations in this gene have been previously associated with human ALL. DeKoter hopes these studies will ultimately lead to improved, less toxic, therapies for . Currently, about 80% of ALL patients go into complete remission when treated with aggressive chemotherapy.

More information: http://bloodjournal.hematologylibrary.org/content/early/2011/07/15/blood-2011-02-335539.abstract

Related Stories

Recommended for you

Breakable genes may promote disease and brain cell diversity

February 11, 2016

Howard Hughes Medical Institute (HHMI) researchers have identified 27 genes in brain stem cells that are prone to a type of DNA damage. The fragility of those genes could explain why they are often mutated or deleted in cancers ...

Common gene variant influences food choices

February 9, 2016

If you're fat, can you blame it on your genes? The answer is a qualified yes. Maybe. Under certain circumstances. Researchers are moving towards a better understanding of some of the roots of obesity.

Timebomb in the testicles investigated

February 8, 2016

Oxford scientists have for the first time been able to identify the origins of some severe disease-causing mutations within the testicles of healthy men. This discovery will help our understanding of how certain serious genetic ...

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.