DNA damage response

and its relevance to human disease


DNA INSTABILITY AND THE DAMAGE RESPONSE SYSTEM

We are surrounded by factors the can cause mutations or damage to our DNA, creating instability in our genes which is detrimental to our survival. Our cells however, work to maintain this natural stability by using a sophisticated process called the DNA damage response (Gartner, A. et al). As every cell is going through its cell cycle, this process monitors the genome for any damage to the DNA due to genotoxic agents such as radiation or mutagenic organic compounds. DNA damage response is carried out by the use of checkpoint proteins which recognize damaged DNA at different stages of the cell cycle and trigger one of two possible outcomes. (Ahmed, S. 2000)



RELEVANCE OF C. ELEGANS TO STUDYING HUMAN DAMAGE RESPONSE SYSTEMS

The level of similarity between the C. elegans genome and the human genome makes it easy to understand why the nematode is a great model for studying the DNA damage response system and the genes responsible for it (DDR genes). In one study, Simon et al. carried out an experiment to find the genomic maps of the DNA damage response in C. elegans. In this study, the researchers analyzed the protein binding capabilities of the DDR genes. They were not only able to identify 12 worm DDR orthologs, but also were able to find as many as 17 novel genes in this species. Experiments were performed to see the phenotypic effect of the absence of each of these genes, which allowed the researchers to better understand the function of the genes identified. Damage to certain genes that gave rise to checkpoint proteins resulted in abnormal phenotypes that increase the organism's susceptibility to development of cancer.



EXAMPLES OF GENES STUDIED IN THIS FIELD

In a detailed study of checkpoint proteins Ahmed et al., while performing cloning experiments using PCR technology identified two checkpoint alleles, rad-5 and clk-2 (Ahmed et. al. 2001). These two genes among many others were found to comprise a great part of the organism's DNA damage response system (Gartner, A, et al 2000), the existence of these proteins was presented and certain characteristics were derived. The most important finding of this study is that the checkpoint pathway is evolutionarily conserved. In other words, a pathway was not generated de novo in the worm. It has been present for many years and is probably present in similar forms in many other organisms, including humans. The great amount of homology between C. elegans and humans makes this hypothesis much more probable (Chalfie, M., 1998)

To read more about polymerase chain reaction, click here.


RELEVANCE TO CANCER

We know that cancer arises from mutations and damage to DNA molecules due to factors such as radiation. These factors cause alterations in our DNA damage response system by disabling the checkpoint proteins, altering the cell cycle arrest or changing the apoptotic pathway. The use of C. elegans as a model for studying these pathways has provided us with an opportunity to learn about human diseases as well. Button et. Al., using protein-protein mapping identified a C. elegans checkpoint protein, CO4F12.3, which is an ortholog of a human gene called hBCL3 (Boulton et. al. 2002). This human gene is often associated with chromosomal translocation and proliferation in cancer cells. It is usually found with damage in a type of human cancer called Chronic Lymphocytic Leukemia, a cancer of blood cells. This introduces the possibility that hBLC3 is also involved in a checkpoint pathway in human cells which would explain its translocation and amplification in Chronic Leukemias.




references

1. Ahmed, S., Alpi, A., Hengartner, M. O., Gartner, A., 2001. C. elegans RAD-5/CLK-2 defines a new DNA damage checkpoint. Curr. Biol. 11(24), 1934-44
2. Boulton, S. J., Gartner, A., Reboul, J., Valgio, P., Dyson, N., Hill, D., Vidal, M., 2002. Combined Functional Genomic Maps of the C. elegans DNA Damage response. Science, 295, 127-131

3. Gartner,A., Milstein, S., Ahmed, S., Hodgkin, J., Hengartner, M.O., 2000. A conserved checkpoint pathway mediates DNA damage-induced apoptosis and cell cycle arrest in C. elegans. Mol. Cell. 5:435-443

4. Kuwabara, P. E., O'Neil, N.,2001. The use of functional genomics in C. elegans for studying human development and disease.J. Inherit. Metab. Dis. 24, 127-138