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Combining\u00a0in vitro biochemical assays, in cellulo molecular biology, and bioinformatic analyses, we investigated how NTHL1 loss affects mitochondrial DNA (mtDNA) stability and mitochondrial function. Contrary to the conventional view that mtDNA damage is solely detrimental, we find that NTHL1 loss confers a beneficial, mitochondria-initiated phenotype in human cells. Despite accumulating mtDNA lesions, NTHL1 loss unexpectedly increases mtDNA copy number, elevates oxidative phosphorylation protein levels, and enhances mitochondrial respiration. NTHL1\u2212\/\u2212\u00a0cells also show increased mitochondrial mass and higher levels of the biogenesis regulator PGC1\u03b1 and the fusion protein OPA1, indicating an adaptive response that boosts mitochondrial function and capacity. Consequently, NTHL1\u2212\/\u2212 cells exhibit resistance to mitochondrial stress, accompanied by increased eIF2\u03b1 phosphorylation and reduced MYC levels, converging on a broader transcriptional adaptive program. This phenotype depends on mitochondrial NTHL1 and reactive oxygen species (ROS) signaling, since treatment with ROS scavengers or mitochondria-specific reintroduction of NTHL1 rescues it. Together, these findings position NTHL1 as a key modulator of mtDNA stability and mitochondrial function, revealing that loss of this DNA repair enzyme shifts cellular metabolism toward a stress-adaptive state and enhances resilience to oxidative stress.<\/jats:p>","DOI":"10.1093\/nar\/gkag876","type":"journal-article","created":{"date-parts":[[2026,9,4]],"date-time":"2026-09-04T11:34:09Z","timestamp":1788521649000},"source":"Crossref","is-referenced-by-count":0,"title":["Functional impact of Nth like DNA glycosylase 1 on mitochondrial dynamics"],"prefix":"10.1093","volume":"54","author":[{"given":"Lisa","family":"Hubers","sequence":"first","affiliation":[{"name":"Department of Microbiology, Oslo University Hospital and University of Oslo , 0424 Oslo ,","place":["Norway"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alexander Myhr","family":"Skjetne","sequence":"additional","affiliation":[{"name":"Department of Microbiology, Oslo University Hospital and University of 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