In humans, the loss-of-function of TREM2 or its coreceptor DAP12 is responsible for the recessively inherited Nasu-Hakola disease (also known as Polycystic lipomembranous osteodysplasia with sclerosing leukoencephalopathy; PLOSL), characterized by an early onset dementia associated with bone cysts [36], [37]. Here, TREM2 is linked to Nasu-Hakola disease.