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Mouse models for ATR deficiency
1Human DNA Damage Response Disorders Group, Genome Damage & Stability Centre, University of Sussex, Falmer, Brighton, East Sussex BN1 9RQ, UK. m.o-driscoll@sussex.ac.uk
Abstract:
ATM and ATR orchestrate overlapping DNA damage responses in reply to different forms of DNA strand discontinuities. But, knockout mouse models suggest that ATR is essential for viability in contrast to ATM. Recently, more sophisticated mouse models have been published including a conditional ATR-knockdown system and by modelling the human ATR-Seckel syndrome-causative mutation. Here, I will overview and contrast these models highlighting the advances both represent in our understanding of how defects in the ATR-dependent DNA damage response can impact on normal development, tissue homeostasis, ageing and cancer.
Insights
Ataxia-telangiectasia mutated (ATM) and ATR kinases manage DNA damage. New mouse models reveal ATR
Area of Science:
- Molecular Biology
- Genetics
- Cellular Biology
Background:
- ATM and ATR proteins are key regulators of DNA damage response pathways.
- These pathways are crucial for maintaining genomic stability.
- Distinct roles for ATM and ATR in cellular viability are suggested by knockout models.
Purpose of the Study:
- To overview and contrast sophisticated mouse models for studying ATR.
- To highlight advances in understanding ATR-dependent DNA damage response.
- To explore the impact of ATR defects on development, homeostasis, aging, and cancer.
Main Methods:
- Review of recent publications on conditional ATR-knockdown mouse models.
- Analysis of mouse models mimicking human ATR-Seckel syndrome mutations.
- Comparative analysis of different mouse models to understand ATR function.
Main Results:
- Conditional ATR-knockdown models offer insights into ATR's essential role.
- ATR-Seckel syndrome models illuminate the consequences of specific ATR mutations.
- These models reveal ATR's critical involvement in development and disease.
Conclusions:
- Sophisticated mouse models have significantly advanced the understanding of ATR's function.
- ATR's role extends beyond DNA repair to development, aging, and cancer.
- Further research using these models will elucidate ATR-dependent pathways.
