Hereditary tyrosinemia type 1 metabolites impair DNA excision repair pathways.

Abstract:

: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.

authors

van Dyk E,Steenkamp A,Koekemoer G,Pretorius PJ

doi

10.1016/j.bbrc.2010.09.002

subject

Has Abstract

pub_date

2010-10-08 00:00:00

pages

32-6

issue

1

eissn

0006-291X

issn

1090-2104

pii

S0006-291X(10)01677-3

journal_volume

401

pub_type

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