deoxyguanosine-triphosphate and MELAS-Syndrome

deoxyguanosine-triphosphate has been researched along with MELAS-Syndrome* in 1 studies

Other Studies

1 other study(ies) available for deoxyguanosine-triphosphate and MELAS-Syndrome

ArticleYear
Role of MHC class I in immune surveillance of mitochondrial DNA integrity.
    Journal of immunology (Baltimore, Md. : 1950), 2003, Apr-01, Volume: 170, Issue:7

    Mitochondrial DNA is subject to increased rates of mutations due to its proximity to the source of reactive oxygen species. Here we show that increased MHC class I (MHC I) expression serves to alert the immune system to cells with mitochondrial mutations. MHC I is overexpressed in fibroblasts with mitochondrial dysfunction from patients with mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes and in lymphocytes from purine nucleoside phosphorylase-deficient immune-deficient mice with mitochondrial DNA deletions. Consistent with a role of MHC I in the elimination of cells containing mitochondrial DNA mutations, mice deficient in MHC I accumulate mitochondrial DNA deletions in various tissues. These observations in both mice and humans suggest a role for the immune system in preventing reversion of mitochondrial DNA back into a parasitic state following deleterious mutations affecting mitochondrial oxidative phosphorylation.

    Topics: Animals; Deoxyguanine Nucleotides; DNA Damage; DNA-Binding Proteins; DNA, Mitochondrial; Fibroblasts; Histocompatibility Antigens Class I; Humans; Immunologic Deficiency Syndromes; Immunologic Surveillance; Interferon-gamma; MELAS Syndrome; Mice; Mice, Inbred C57BL; Mice, Knockout; Osteosarcoma; Phosphorylation; Purine-Nucleoside Phosphorylase; Reactive Oxygen Species; STAT1 Transcription Factor; Trans-Activators; Tumor Cells, Cultured

2003