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Such a downregulation was also observed at OGG1, MYH, and APE1 protein levels.

We found that XP-C fibroblasts are characterized by downregulated expression of different BER factors including OGG1, MYH, APE1, LIG3, XRCC1, and Polβ. Since base excision repair (BER) is the primary regulator of oxidative DNA damage, we characterized, post-Ultraviolet B-rays (UVB)-irradiation, the detailed effect of three different XPC mutations in primary fibroblasts derived from XP-C patients on mRNA, protein expression and activity of different BER factors. Xeroderma Pigmentosum C (XPC) is a multi-functional protein that is involved not only in the repair of bulky lesions, post-irradiation, via nucleotide excision repair (NER) per se but also in oxidative DNA damage mending.

5Centre de Référence pour les Maladies Rares de la Peau, CHU de Bordeaux, Bordeaux, France.

4Université de Bordeaux, Inserm, BMGIC, U1035, Bordeaux, France.

3University Grenoble Alpes, CEA, Inserm, BIG-BGE U1038, Grenoble, France.

2Laboratory of Cancer Biology and Molecular Immunology, Faculty of Sciences I, Lebanese University, Hadath, Lebanon.

1University Grenoble Alpes, SyMMES/CIBEST UMR 5819 UGA-CNRS-CEA, Grenoble, France.

Nour Fayyad 1, Farah Kobaisi 1,2,3, David Beal 1, Walid Mahfouf 4, Cécile Ged 4,5, Fanny Morice-Picard 5, Mohammad Fayyad-Kazan 2, Hussein Fayyad-Kazan 2, Bassam Badran 2, Hamid R.