Hereditary tyrosinemia type 1 metabolites impair DNA excision repair pathways
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作者:
van Dyk, E.
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North West Univ, Sch Phys & Chem Sci, Ctr Human Metabon, ZA-2520 Potchefstroom, South AfricaNorth West Univ, Sch Phys & Chem Sci, Ctr Human Metabon, ZA-2520 Potchefstroom, South Africa
van Dyk, E.
[1
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Steenkamp, A.
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North West Univ, Sch Phys & Chem Sci, Ctr Human Metabon, ZA-2520 Potchefstroom, South AfricaNorth West Univ, Sch Phys & Chem Sci, Ctr Human Metabon, ZA-2520 Potchefstroom, South Africa
Steenkamp, A.
[1
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Koekemoer, G.
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North West Univ, Stat Consultat Serv, ZA-2520 Potchefstroom, South AfricaNorth West Univ, Sch Phys & Chem Sci, Ctr Human Metabon, ZA-2520 Potchefstroom, South Africa
Koekemoer, G.
[2
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Pretorius, P. J.
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North West Univ, Sch Phys & Chem Sci, Ctr Human Metabon, ZA-2520 Potchefstroom, South AfricaNorth West Univ, Sch Phys & Chem Sci, Ctr Human Metabon, ZA-2520 Potchefstroom, South Africa
Pretorius, P. J.
[1
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[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
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.
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North West Univ, Ctr Human Metabon, Sch Phys & Chem Sci, ZA-2520 Potchefstroom, South AfricaNorth West Univ, Ctr Human Metabon, Sch Phys & Chem Sci, ZA-2520 Potchefstroom, South Africa
van Dyk, E.
Pretorius, P. J.
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North West Univ, Ctr Human Metabon, Sch Phys & Chem Sci, ZA-2520 Potchefstroom, South AfricaNorth West Univ, Ctr Human Metabon, Sch Phys & Chem Sci, ZA-2520 Potchefstroom, South Africa
机构:
King Faisal Specialist Hosp & Res Ctr, Dept Med Genet, Riyadh 11211, Saudi ArabiaKing Faisal Specialist Hosp & Res Ctr, Dept Med Genet, Riyadh 11211, Saudi Arabia
Al-Sayed, MA
Al-Asmari, AM
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King Faisal Specialist Hosp & Res Ctr, Dept Med Genet, Riyadh 11211, Saudi ArabiaKing Faisal Specialist Hosp & Res Ctr, Dept Med Genet, Riyadh 11211, Saudi Arabia
Al-Asmari, AM
Rashed, MS
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King Faisal Specialist Hosp & Res Ctr, Dept Med Genet, Riyadh 11211, Saudi ArabiaKing Faisal Specialist Hosp & Res Ctr, Dept Med Genet, Riyadh 11211, Saudi Arabia