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CHOP experts collaborate in gene survey of childhood brain cancer; intriguing clues found
Date:12/16/2010

gehog and Wnt signaling pathways, the current study also identified new and unexpected mutations in the MLL2 and MLL3 genes, genes not previously implicated in medulloblastoma. Both are tumor suppressor genes, genes that normally act as a brake on the development of cancer. If a mutation perturbs such a gene, cancer may then develop.

"Like other genes found in medulloblastoma, the MLL2 and MLL3 genes disrupt normal brain development during childhood," said co-author Peter C. Phillips, M.D., director of the Pediatric Neuro-oncology Program at The Children's Hospital of Philadelphia. "These genes interfere with biological signals during development, specifically with a process called histone methylation." In histone methylation, chemical tags that bind to DNA regulate how specific genes become active.

Both Curran and Phillips stressed the importance of collaboration among many institutions to the success of this study. "Even the largest oncology programs may see small numbers of medulloblastoma patients, so sharing data and materials is crucial to producing stronger statistical results," said Phillips. Children's Hospital and other centers have contributed resources and data to the Children's Brain Tumor Tissue Consortium, which provided tumor samples to the current study.

Direct clinical applications from this study are not immediate, but both researchers noted that clinical trials are already in progress to test whether inhibiting the Hedgehog pathway benefits medulloblastoma patients. Phillips added that the current study may lay a foundation for developing drugs that target the gene pathways associated with the MLL2 and MLL3 genes.


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Contact: John Ascenzi
Ascenzi@email.chop.edu
267-426-6055
Children's Hospital of Philadelphia
Source:Eurekalert

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