ATM and Huntington disease: Among the numerous molecular interactions and signaling pathways implicated in HD pathomechanism, transcriptional dysregulation (Jimenez-Sanchez et al., 2017; Ross and Tabrizi, 2011; Valor, 2015), mitochondrial (mt) dysfunction (Shirendeb et al., 2011; Siddiqui et al., 2012), DNA strand break accumulation, and atypical ataxia telangiectasia-mutated (ATM) pathway activation, involved in the DNA damage response (Bertoni et al., 2011; Giuliano et al., 2003; Illuzzi et al., 2009; Xh et al., 2014), have emerged as key players in HD-related neuronal dysfunction.