It should be noted that CSPalpha aggregation in Alzheimer’s disease, where there is no known genetic link, would be different from CSPalpha aggregation in adult-onset neuronal ceroid lipofuscinosis (ANCL), which is caused by missense mutations in the DNAJC5 gene.68,69 In ANCL, mutant CSPalpha aggregates mislocalize intracellularly, which results in CSPalpha exclusion from synapses.69,70 However, in Alzheimer’s disease brain, we did not find any evidence for intracellular accumulation of CSPalpha or for its exclusion from intact, synaptophysin-containing synapses. This evidence concerns the gene DNAJC5 and early-onset autosomal dominant Alzheimer disease.