Abstract
Telomere length has been proposed as a biomarker of biological age and a risk factor for age-related diseases and cancer. Substantial progress has been made in recent decades in understanding the complex molecular relationships in this research field. However, the majority of telomere studies have been conducted in adults. The data on telomere dynamics in pediatric cancers is limited, and interpretation can be challenging, especially in cases where results are contrasting to those in adult entities. This review describes recent advances in the molecular characterization of structure and function of telomeres, regulation of telomerase activity in cancer pathogenesis in general, and highlights the key advances that have expanded our views on telomere biology in pediatric cancer, with special emphasis on the central role of telomere maintenance in neuroblastoma. Furthermore, open questions in the field of telomere maintenance research are discussed in the context of recently published literature.
Highlights
Precise control of genomic stability is essential for cellular integrity and tissue homeostasis in multicellular organisms
This review describes recent advances in the molecular characterization of structure and function of telomeres, regulation of telomerase activity in cancer pathogenesis in general, and highlights the key advances that have expanded our views on telomere biology in pediatric cancer, with special emphasis on the central role of telomere maintenance in neuroblastoma
Still, deciphering the complexity of telomere biology is steadily increasing our knowledge of how telomeres function to maintain genome integrity and how their dysfunction gives rise to human disease
Summary
Precise control of genomic stability is essential for cellular integrity and tissue homeostasis in multicellular organisms. Telomere biology has been implicated in major human biological processes and conditions, such as reproduction, aging, cancer, cardiovascular disorders, various heritable syndromes, other human pathologies and even mental health [4,5,6], and many excellent comprehensive reviews on these topics are available, summarizing current knowledge and outlining promising future trends [4,5,7,8]. Still, deciphering the complexity of telomere biology is steadily increasing our knowledge of how telomeres function to maintain genome integrity and how their dysfunction gives rise to human disease. We focus on the latest progress in understanding the molecular nature of telomere biology in cancer with particular emphasis on pediatric oncology, and highlight promising findings that may provide the basis for development of molecular diagnosis and novel approaches to rational treatment strategies
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