Promising Treatment Strategy can Fight Deadly Brain Tumor in Children

by Iswarya on March 4, 2019 at 3:47 PM

New study finds that an inhibitor of an enzyme known as ACVR1 slows tumor growth and extends survival in an animal model of diffuse intrinsic pontine glioma (DIPG), a fatal brain tumor in kids. The findings of the study are published in the journal Nature Communications.

"Our results are encouraging and suggest that it might be reasonable to test an inhibitor of this enzyme in a clinical trial," says senior author Oren Becher, MD, from Stanley Manne Children's Research Institute at Ann & Robert H. Lurie Children's Hospital of Chicago and Associate Professor of Pediatrics at Northwestern University Feinberg School of Medicine. "Before that, we need to evaluate different ACVR1 inhibitors in animal models to make sure we bring the safest and effective agent to trials with children."

Brain Tumor in Children

An abnormal growth of cells in the brain is called brain tumor. Most tumors arise within the brain (primary). They can be benign or cancerous.

‘ACVR1, an inhibitor of an enzyme slows tumor growth and prolongs survival in an animal model of diffuse intrinsic pontine glioma (DIPG), a life-threatening brain tumor in kids.’
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In 2014, Dr. Becher's lab co-discovered that ACVR1 mutations are found in approximately 25 percent of DIPGs, leading the enzyme to be overactive.

In the current study, Dr. Becher and colleagues demonstrate for the first time in an animal model that this enzyme mutation cooperates with a histone mutation (H3.1 K27M) found in 20 percent of DIPGs. Together, these mutations are important in initiating tumor development.

Brain Tumor in Children: New Therapy Target Offers Hope

Diffuse Intrinsic Pontine Glioma (DIPG) is a type of brain tumor in children. Restoring the p16 gene could help in treatment.

Histone is a protein that acts as a spool for DNA, helping to package the six-foot-long DNA strand into the tiny nucleus of every cell. Histones also help regulate which genes turn on and off, a process that goes awry when there is a histone mutation.

"Our future work will examine why and how the ACVR1 and histone mutations work together to trigger DIPG development," says Dr. Becher, who also is the Rory David Deutsch Malignant Brain Tumor Research Scholar. "Greater insight into this process will bring us closer to identifying a successful therapy for children with DIPG."

Source: Eurekalert

Glioma Brain Tumor

Democrat Senator Edward Kennedy has been diagnosed with Glioma, a kind of brain tumor. Tests carried out on the 76 -year old politician revealed a tumor on his left parietal lobe.

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