In humans, highly related homologues of l(2)gl, LLGL1, and LLGL2 have been identified, mapping to the short and long arm of chromosome 17. LLGL1 has been located in a critical pericentromeric region, 17p11.2-12 containing cancer susceptibility genes for primitive neuroectodermal tumors [21]. The gene discussed is LLGL1; the disease is primitive neuroectodermal tumor.