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In page Mitochondrial DNA:

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The DNA oxidation product 8-oxoguanine (8-oxoG) is a well-established marker of oxidative DNA damage. In persons with amyotrophic lateral sclerosis (ALS), the enzymes that normally repair 8-oxoG DNA damages in the mtDNA of spinal motor neurons are impaired.[6] Thus oxidative damage to mtDNA of motor neurons may be a significant factor in the etiology of ALS.[citation needed]