Given the established role of MCL1 as a critical pro-survival factor in a range of hematologic malignancies, including MM, AML, and NHL [26,27,115,116,117], and a resistance factor to BH3 mimetics targeting BCL2 and BCLxL [118], there has been longstanding interest in designing potent and selective MCL1 inhibitors for therapeutic use. This evidence concerns the gene MCL1 and Miyoshi myopathy.