Unraveling why children with Down syndrome have increased leukemia risk

February 22, 2012

Children with Down syndrome (DS) have an increased risk of developing leukemia, in particular acute megakaryoblastic leukemia (AMKL) and acute lymphoblastic leukemia (ALL). Through their studies in a mouse model of DS, a team of researchers led by John Crispino, at Northwestern University, Chicago, has now identified a potential explanation as to why children with DS are at increased risk of AMKL. In doing so, they have also identified a candidate therapeutic target.

DS is a genetic condition in which a person has an extra copy of (they have 3 copies rather than 2). It is not clear, however, which genes on chromosome 21 are responsible for the increased risk of developing leukemia observed in children with DS. Crispino and colleagues found that increased expression of the protein templated by the chromosome 21 gene Dyrk1a promotes AMKL in a mouse model of DS. Interestingly, an inhibitor of DYRK1A activity inhibited the in vitro growth of AMKL cells lines from individuals with DS. Crispino and colleagues therefore suggest that developing small-molecule inhibitors of DYRK1A activity may have therapeutic potential for DS-AMKL.

Shai Izraeli and Yehudit Birger, at Sheba Medical Center, Israel, second this idea in an accompanying commentary.

Explore further: Cells involved with Down syndrome restored

More information: Increased dosage of the chromosome 21 ortholog Dyrk1a promotes megakaryoblastic leukemia in a murine model of Down syndrome. View this article at: www.jci.org/articles/view/60455?key=ebdcc7eb0722a8d27c0b

DYRK1A in Down Syndrome: an oncogene or tumor suppressor? View this article at: www.jci.org/articles/view/62372?key=3ae146d8c86858ca1679

Related Stories

Cells involved with Down syndrome restored

January 24, 2006

Johns Hopkins University scientists in Baltimore say they've restored the normal growth of nerve cells in the brains of mouse models of Down syndrome.

Nintendo sells 100 million DS consoles

March 12, 2009

Nintendo said sales of its handheld DS have topped 100 million machines less than five years since its launch, reaching the milestone faster than any other game console.

Why do people with Down syndrome have less cancer?

May 20, 2009

Most cancers are rare in people with Down syndrome, whose overall cancer mortality is below 10 percent of that in the general population. Since they have an extra copy of chromosome 21, it's been proposed that people with ...

New drug for children with high-risk leukemia

July 28, 2009

Each year, approximately 4,500 children in America are diagnosed with leukemia, according to the Leukemia and Lymphoma Society. A potentially deadly cancer of the blood, it is the most common cancer in children.

Key player found for a cancer typical in Down syndrome

March 1, 2010

Between 5 and 10 percent of babies with Down syndrome develop a transient form of leukemia that usually resolves on its own. However, for reasons that haven't been clear, 20 to 30 percent of these babies progress to a more ...

Gene loss causes leukemia: study

May 17, 2010

Researchers from VIB and K.U.Leuven, both in Flanders, Belgium, have discovered a new factor in the development of acute lymphoblastic leukemia, a disease that mainly affects children. In the cells of the patients, the specific ...

Recommended for you

Solving the mystery of meningiomas reveals a surprise twist

August 23, 2016

In solving one mystery—the genetic roots of benign brain tumors called meningiomas—a team of scientists led by Yale researchers stumbled upon an even greater one: How is it possible that two of the mutations linked to ...

Two key proteins preserve vital genetic information

August 22, 2016

Cancer is often driven by various genetic mutations that are acquired through changes to a person's DNA over time. These alterations can occur at the chromosome level if the proteins are not properly organized and segregated ...

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.