This gene encodes a membrane protein that forms a receptor signaling complex with the TYRO protein tyrosine kinase binding protein. It is involved in immune response and in chronic inflammation. Defects in this gene are a cause of polycystic lipomembranous osteodysplasia with sclerosing leukoencephalopathy (PLOSL). Inactivating mutations of TREM2 have been associated with an autosomal recessive form of early-onset dementia in literature. This evidence concerns the gene TYROBP and Nasu-Hakola disease.