These data show elevated mitophagy marker expression specifically in SRSF2-mutant MDS and AML, and we therefore focused on the impact of SRSF2P95H on mitochondrial function and the role of GSK-3–dependent PINK1 splicing in the regulation of mitophagy in SRSF2P95H/+ cells. Here, SRSF2 is linked to myelodysplastic syndrome.