The use of Bap1 knockout models has shown that heterozygosity in the germline predisposes to asbestos-induced MM (53, 59), and similar results were obtained with two knock-in models (54) that harbored different germline mutations that were identical to the ones found in two BAP1 tumor predisposition syndrome (BAP1-TPDS) families that exhibited a very high incidence of MM (8). Here, BAP1 is linked to Miyoshi myopathy.