Indeed, factors involved in messenger ribonucleoprotein (mRNP) biogenesis, like the THO complex, TDP‐43 or SRSF1 [19, 20, 21, 22], DNA topoisomerase I and II [23, 24], DNA:RNA hybrid resolvases like ribonucleases H1 (RNH1), 2 (RNH2) and DICER [9, 25, 26], DNA:RNA helicases like DDX39b/UAP56, DDX5, DDX47, DDX21 or DHX9 and others [27, 28, 29, 30, 31, 32], and DNA repair factors such as BRCA1, BRCA2, the MRN complex, and those of the Fanconi anemia (FA) pathway [4, 7–9, 33] have been shown to actively prevent R‐loop accumulation and R‐loop‐mediated GIN. Here, DDX39B is linked to Friedreich ataxia.