ABCB7 is a mitochondrial ATP-binding cassette transporter that exports glutathione-coordinated iron-sulfur clusters, particularly [2Fe-2S]-(GS)4 clusters, from mitochondria to the cytosol in an ATP-dependent manner 123. This export is essential for cytosolic iron-sulfur cluster-containing protein assembly and iron homeostasis 4. ABCB7 also functions within a complex with FECH and ABCB10 to regulate heme biosynthesis and mitochondrial function 5. The protein stabilizes ferrochelatase, the enzyme incorporating iron into protoporphyrin IX during heme synthesis 6. Mutations in ABCB7 cause X-linked sideroblastic anemia with cerebellar ataxia (XLSA/A) 47. In myelodysplastic syndromes with ring sideroblasts (MDS-RS), SF3B1 mutations induce ABCB7 mis-splicing and downregulation, leading to impaired iron-sulfur cluster export and disrupted heme synthesis 86. This ABCB7 deficiency creates vulnerability to copper ionophore-induced cell death (cuproptosis), presenting a therapeutic opportunity in SF3B1-mutated acute myeloid leukemia 9. Beyond hematologic malignancies, ABCB7 knockdown inhibits esophageal cancer progression by suppressing TGF-β/Smad signaling and inducing cell death 10. In fish models, ABCB7 mutations cause abnormal iron metabolism and hepatic lipid accumulation 11.