This is specifically associated with the inactivation of the H2AK119ub1 specific de-ubiquitinase BAP1 that occurs with high frequency in uveal melanoma (∼45%) and mesothelioma (∼22%) as well as in several other tumor types with lower frequencies like atypical Spitz tumors (∼11%), clear cell renal cell carcinoma (∼18%), ovary (∼5%), and colon-rectum (∼3%) (Carbone et al., 2013). The gene discussed is BAP1; the disease is neoplasm.