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Ataxia telangiectasia is caused by mutations in ATM and represents a paradigm for cancer predisposition and neurodegenerative syndromes linked to deficiencies in the DNA-damage response. The role of ATM as a key regulator of signalling following DNA double-strand breaks (DSBs) has been dissected in extraordinary detail, but the impact of this process on DSB repair still remains controversial. Here we develop novel genetic and molecular tools to modify the structure of DSB ends and demonstrate that ATM is indeed required for efficient and accurate DSB repair, preventing cell death and genome instability, but exclusively when the ends are irreversibly blocked. We therefore identify the nature of ATM involvement in DSB repair, presenting blocked DNA ends as a possible pathogenic trigger of ataxia telangiectasia and related disordersWork in F.C.-L. laboratory is funded with grants from the Spanish Government (SAF2010-21017 and BFU2010-11042-E, Ministerio de Ciencia e Innovación), the regional Andalusian Government (CVI-7948) and the European Union (PERG07-2010-268466) and with the following fellowships: Formación Personal Investigador (BES-2011-047351, Ministerio de Ciencia e Innovación) for A.A.-Q., Beca Predoctoral AEFAT (Asociación Española Familia Ataxia Telangiectasia) for A.S.-B., Personal Investigador en Formación (Universidad de Sevilla) for J.A.L. and Ramón y Cajal (RYC-2009-03928, Ministerio de Ciencia e Innovación) for F.C.-L. L.M.E. is supported by the Miguel Servet program, (Instituto Carlos III) and the Spanish Government grant (BFU2011-25734, Ministerio de Ciencia e Innovación)Peer Reviewe

ATM specifically mediates repair of double-strand breaks with blocked DNA ends