Hereditary tyrosinemia type 1 metabolites impair DNA excision repair pathways

被引：15
|
作者：
van Dyk, E. [1 ]
Steenkamp, A. [1 ]
Koekemoer, G. [2 ]
Pretorius, P. J. [1 ]
机构：
[1] North West Univ, Sch Phys & Chem Sci, Ctr Human Metabon, ZA-2520 Potchefstroom, South Africa
[2] North West Univ, Stat Consultat Serv, ZA-2520 Potchefstroom, South Africa
来源：
BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS | 2010年 / 401卷 / 01期
基金：
新加坡国家研究基金会;
关键词：
Hereditary tyrosinemia; Base excision repair; Nucleotide excision repair; Comet assay; COMET ASSAY; DAMAGE; FUMARYLACETOACETATE; HOGG1;
D O I：
10.1016/j.bbrc.2010.09.002
中图分类号：
Q5 [生物化学]; Q7 [分子生物学];
学科分类号：
071010 ; 081704 ;
摘要：
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.
引用
收藏
页码：32 / 36
页数：5
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