The importance of TREM2 in neuronal health was first demonstrated by genetic studies that identified TREM2 variants in families with NHD (also known as polycystic lipomembranous osteodysplasia with sclerosing leukoencephalopathy or PLOSL), a fatal disease characterized by presenile dementia and bone cysts (Paloneva et al., 2001, 2002; Klünemann et al., 2005; Bianchin et al., 2006). The gene discussed is TREM2; the disease is bone disorder.