Unknown

Dataset Information

0

Targeting the DNA Damage Response for the Treatment of High Risk Neuroblastoma.


ABSTRACT: Despite intensive multimodal therapy, the survival rate for high risk neuroblastoma (HR-NB) remains <50%. Most cases initially respond to treatment but almost half will subsequently relapse with aggressive treatment resistant disease. Novel treatments exploiting the molecular pathology of NB and/or overcoming resistance to current genotoxic therapies are needed before survival rates can significantly improve. DNA damage response (DDR) defects are frequently observed in HR-NB including allelic deletion and loss of function mutations in key DDR genes, oncogene induced replication stress and cell cycle checkpoint dysfunction. Exploiting defects in the DDR has been a successful treatment strategy in some adult cancers. Here we review the genetic features of HR-NB which lead to DDR defects and the emerging molecular targeting agents to exploit them.

SUBMITTER: Southgate HED 

PROVIDER: S-EPMC7145987 | biostudies-literature | 2020

REPOSITORIES: biostudies-literature

altmetric image

Publications

Targeting the DNA Damage Response for the Treatment of High Risk Neuroblastoma.

Southgate Harriet E D HED   Chen Lindi L   Curtin Nicola J NJ   Tweddle Deborah A DA  

Frontiers in oncology 20200403


Despite intensive multimodal therapy, the survival rate for high risk neuroblastoma (HR-NB) remains <50%. Most cases initially respond to treatment but almost half will subsequently relapse with aggressive treatment resistant disease. Novel treatments exploiting the molecular pathology of NB and/or overcoming resistance to current genotoxic therapies are needed before survival rates can significantly improve. DNA damage response (DDR) defects are frequently observed in HR-NB including allelic de  ...[more]

Similar Datasets

| S-EPMC6535646 | biostudies-literature
| S-EPMC6162495 | biostudies-literature
| S-EPMC7452577 | biostudies-literature
| S-EPMC4317796 | biostudies-literature
| S-EPMC9282716 | biostudies-literature
| S-EPMC8347825 | biostudies-literature
| S-EPMC4990925 | biostudies-literature
| S-EPMC7663807 | biostudies-literature
| S-EPMC7201617 | biostudies-literature
| S-EPMC6696225 | biostudies-literature