A portion of amino acids 1-200 was used as the immunogen for the TREM2 antibody.
Polycystic lipomembranous osteodysplasia with sclerosing leukoencephalopathy (PLOSL), known as Nasu-Hakola disease , is a recessively inherited disease where individuals display early-onset progressive dementia and bone cysts, which leads to death. Mutations in TYROBP (DAP12), which codes for a membrane receptor component in natural-killer and myeloid cells and mutations in triggering receptor expressed on myeloid cells-2 (TREM-2), correlate well to the pathology of PLOSL. TREM-2 is a cell surface receptor on human monocyte-derived dendritic cells that forms a receptor signaling complex with DAP12 and triggers activation of the immune response in macrophages and dendritic cells (DC). The TREM-2/DAP12 complex is a molecular promoter of upregulation of CC chemokine receptor 7, partial DC maturation, and DC survival through activation of protein tyrosine kinases and extracellular signal regulated kinase. The human chronic inflammatory TREM-2 gene maps to chromosome 6p21.1 and encodes a 230 amino acid protein.
