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- W3171670720 abstract "The Xeroderma pigmentosum group A (XPA) protein plays critical roles in nucleotide excision repair pathway (NER), and patients with XPA deficiency exhibit sun sensitivity, high skin cancer predisposition, and many have progressive neurological disorders. Based on a clinical database that we have established (Scheibye-Knudsen et al, submitted) we speculated that there might be mitochondrial dysfunction in individuals with XPA. We therefore investigated mitochondrial function in XPA deficient cells, in primary fibroblasts from patients with XPA and compared these to controls and to cell lines complemented with the normal XPA gene. Microarray analysis indicated increased mitochondrial metabolism in XPA deficient cells, and flow cytometry results showed an increase in mitochondrial content. Cellular and mitochondrial reactive oxygen species (ROS) and the mitochondrial membrane potential (MMP) were increased in XPA cells. These cells also had increased numbers of damaged mitochondria and a deficiency in mitophagy after mitochondrial stress. These findings uncover a novel mitochondrial defect in XPA which could be an important factor in the clinical phenotype. We discuss the dysregulation of important mitochondrial proteins in XPA and propose a mechanism." @default.
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- W3171670720 date "2013-04-01" @default.
- W3171670720 modified "2023-09-23" @default.
- W3171670720 title "Xeroderma pigmentosum group A protein modulates mitophagy through regulation of mitochondrial‐associated proteins" @default.
- W3171670720 doi "https://doi.org/10.1096/fasebj.27.1_supplement.lb468" @default.
- W3171670720 hasPublicationYear "2013" @default.
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