Telomere biology of pediatric cancer.

Abstract:

:One of the hallmarks of cancer is limitless proliferative capacity, which is tightly associated with the ability to maintain telomeres. Over the last decade, the telomere biology of pediatric cancers has begun to be elucidated. Most pediatric leukemias and embryonal solid tumors activate the enzyme telomerase, a specialized reverse transcriptase that adds nucleotide repeats to telomeres. In general, high levels of tumor telomerase expression are associated with unfavorable outcome, although results vary according to tumor type. Some pediatric tumors, including osteosarcoma and glioblastoma multiforme, lack telomerase activity and maintain telomeres via a recombination-based mechanism called ALT (alternative lengthening of telomeres). Telomerase is a highly attractive therapeutic target for pediatric cancer because the enzyme plays a key role in conferring cellular immortality, is present in most tumors, and is relatively specific for cancer cells. Telomerase inhibitors have been evaluated in preclinical models of adult cancers, but few studies have been conducted on pediatric cancers. Further research is required to define how telomere biology can be used to clinical advantage in malignancies of childhood.

journal_name

Cancer Invest

journal_title

Cancer investigation

authors

Tabori U,Dome JS

doi

10.1080/07357900701208683

subject

Has Abstract

pub_date

2007-04-01 00:00:00

pages

197-208

issue

3

eissn

0735-7907

issn

1532-4192

pii

779065502

journal_volume

25

pub_type

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