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Aag-initiated base excision repair drives alkylation-induced retinal degeneration in mice.

Meira, LB, Moroski-Erkul, CA, Green, SL, Calvo, JA, Bronson, RT, Shah, D and Samson, LD (2009) Aag-initiated base excision repair drives alkylation-induced retinal degeneration in mice. Proc Natl Acad Sci U S A, 106 (3). pp. 888-893.

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Vision loss affects >3 million Americans and many more people worldwide. Although predisposing genes have been identified their link to known environmental factors is unclear. In wild-type animals DNA alkylating agents induce photoreceptor apoptosis and severe retinal degeneration. Alkylation-induced retinal degeneration is totally suppressed in the absence of the DNA repair protein alkyladenine DNA glycosylase (Aag) in both differentiating and postmitotic retinas. Moreover, transgenic expression of Aag activity restores the alkylation sensitivity of photoreceptors in Aag null animals. Aag heterozygotes display an intermediate level of retinal degeneration, demonstrating haploinsufficiency and underscoring that Aag expression confers a dominant retinal degeneration phenotype.

Item Type: Article
Divisions : Faculty of Health and Medical Sciences > School of Biosciences and Medicine > Department of Biochemical Sciences
Authors :
Meira, LB
Moroski-Erkul, CA
Green, SL
Calvo, JA
Bronson, RT
Shah, D
Samson, LD
Date : 20 January 2009
DOI : 10.1073/pnas.0807030106
Uncontrolled Keywords : Alkylating Agents, Animals, Apoptosis, DNA Glycosylases, DNA Modification Methylases, DNA Repair, DNA Repair Enzymes, Methyl Methanesulfonate, Methylnitrosourea, Mice, Photoreceptor Cells, Vertebrate, Retinal Degeneration, Tumor Suppressor Proteins
Depositing User : Symplectic Elements
Date Deposited : 28 Mar 2017 14:58
Last Modified : 31 Oct 2017 14:40

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