IRCCS Mondino Foundation
Published July 24, 2023 | Version v1
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Metformin treatment reduces oxidative stress state of RNASEH2-mutated Aicardi-Goutières patients-derived Lymphoblastoid Cell Lines

Authors/Creators

  • 1. IRCCS Mondino Foundation, Pavia (Italy)
  • 2. University of Pavia, Pavia (Italy)
  • 3. University of Pavia, Pavia (Italy); IRCCS Mondino Foundation, Pavia (Italy)

Description

The database includes the raw data of the article “Metformin treatment reduces oxidative stress state of RNASEH2-mutated Aicardi-Goutières patients-derived Lymphoblastoid Cell Lines”. Aim of this work was was to examine the impact of metformin treatment on the oxidative stress state of lymphoblastoid cell lines (LCLs) carrying mutation in RNASEH2B and RNASEH2A AGS related genes and healthy control. The database contains the data obtained by the following evaluations: a) Flow cytometry analysis for the evaluation of ROS release; b) Immunofluorescence for the evaluation of the stress-induced DNA damage by the quantification of the 8-OxoG level and changes of altered membrane potential by the quantification of monomeric and aggregated JC-1 dye; c) RT-qPCR for the evaluation of the expression level of FOXO3-related pathway.

We demonstrated that metformin is able to highly reduce ROS overproduction especially in RNASEH2B-mutated LCLs, to restore the impaired mitochondrial membrane potential and strongly reduce the oxidative DNA damage by reducing the 8-oxoG level in AGS patients. We demonstrated that the beneficial effect of metformin associate with the upregulation of FOXO3-related signaling pathway. Metformin treatment seem to represents a possible therapeutic approach, useful to ameliorate the oxidative stress-related inflammatory condition observed in AGS subjects.

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