화학공학소재연구정보센터
Biochemical and Biophysical Research Communications, Vol.401, No.1, 32-36, 2010
Hereditary tyrosinemia type 1 metabolites impair DNA excision repair pathways
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. (C) 2010 Elsevier Inc. All rights reserved.