An atypical ANCA, anti-human lysosome-associated membrane protein-2 (anti-LAMP-2) antibody, was first identified in patients with pauci-immune glomerulonephritis (GN) in 1995 and has 100% sequence homology with FimH, a bacterial adhesion protein on Gram-negative bacteria. The gene discussed is LAMP2; the disease is ganglioneuroma.