Notably, the known interacting proteins of BTRR that we detected with BioID include components of the Fanconi anemia (FA) complex (FANCM, FANCD2, BRIP1/FANCJ, and CENPX) involved in double-strand break repair and DNA replication stress response (Meetei et al, 2003), double-strand break repair proteins (RAD50 (Franchitto and Pichierri, 2002) and BRCA1 (Acharya et al, 2014)), and proteins that repair stalled replication forks (TOPBP1 (Wang et al, 2013a), ETAA1 (Bass et al, 2016), WRN (Sturzenegger et al, 2014)). The gene discussed is TOPBP1; the disease is Friedreich ataxia.