Biallelic germline inactivation of the MRE11 gene results in an autosomal recessive Ataxia-telangiectasia-like disorder (ATLD1; OMIM: #604391) that, together with ataxia-telangiectasia (AT; OMIM: #208900; caused by ATM inactivation), belongs to spinocerebellar ataxias characterized by disturbances of eye movement or oculomotor apraxia and DNA damage hypersensitivity [79,81,82,83] (Table 1). Here, MRE11 is linked to ataxia telangiectasia.