Hereditary tyrosinemia type 1 metabolites impair DNA excision repair pathways
Date
2010Author
Van Dyk, E.
Steenkamp, A.
Koekemoer, G.
Pretorius, P.J.
Metadata
Show full item recordAbstract
Hereditary tyrosinemia type 1 is an autosomal recessive metabolic disorder, which is caused by a defective fumarylacetoacetate hydrolase enzyme, and consequently metabolites such as succinylacetone and p-hydroxyphenylpyruvate accumulate. We used a modified comet assay to determine the effect of these metabolites on base- and nucleotide excision repair pathways. Our results indicate that the metabolites affected the repair mechanisms differently, since the metabolites had a bigger detrimental effect on BER than on NER
URI
http://hdl.handle.net/10394/5775https://doi.org/10.1016/j.bbrc.2010.09.002
https://www.sciencedirect.com/science/article/pii/S0006291X10016773