As for the precise change of CSPα levels, Henderson and collaborators found that 0.66-fold significantly reduced CSPα levels in patients ANCL by using either a DNAJC5 p.L115R or a DNAJC5 p.L116δ mutation (Henderson et al., 2016), which is in line with other research that measured CSPα levels in brain samples from an advanced ANCL patient with a DNAJC5 p.L116δ mutation and found a 50% reduction (Donnelier et al., 2015), indicating that ANCL occurring in humans is the result of reduced levels of CSPα. This evidence concerns the gene DNAJC5 and adult neuronal ceroid lipofuscinosis.