In human and animal CLN6 disease, and in other forms of NCL, a major component of the storage material is the small subunit c protein of the inner mitochondrial membrane protein ATP synthase (Palmer et al. 1989; Fearnley et al. 1990; Ansari et al. 1995; Kominami et al. 1995; Tyynelä et al. 1997; Umehara et al. 1997; Jolly and Walkley 1999; Ranta et al. 2001; Cook et al. 2002; Cao et al. 2011; Palmer et al. 2013). Here, CLN6 is linked to neuronal ceroid lipofuscinosis.