ATP-binding cassette sub-family B member 10, mitochondrial (ABCB10)

ABCB10 (ATP-binding cassette sub-family B member 10, mitochondrial) is a mitochondrial membrane transporter belonging to the ATP-binding cassette (ABC) transporter superfamily[1][3]. It plays an essential role in exporting physiological substrates—recently identified as biliverdin—from the mitochondrial matrix, supporting erythropoiesis and protecting cells from oxidative stress[3][5]. The protein stabilizes mitoferrin-1, which is crucial for mitochondrial iron uptake and hemoglobin biosynthesis[3][5]. The crystal and cryo-EM structures reveal that ABCB10 forms a homodimer with each monomer containing six transmembrane domains and a nucleotide binding domain; it adopts inward-facing and wide-open conformations, awaiting substrate binding and ATP hydrolysis to drive transport[1][3][5]. ABCB10 is indispensable for red blood cell development, and its loss leads to severe anemia and embryonic lethality in mice[1]. It is considered a potential therapeutic target in diseases involving mitochondrial oxidative stress and iron metabolism disorders, but drug modulators have not been clinically developed[1][3][5].

Other names

ABC-me proteinABC transporter 10 proteinM-ABC2EST20237MTABC2ABC-mitochondrial erythroid proteinATP-binding cassette transporter 10Mitochondrial ATP-binding cassette 2

Hydrolysis of ATP powers substrate transport across mitochondrial inner membrane. ATPase activity can be inhibited by vanadate. Substrate binding triggers conformational change, ATP hydrolysis, and substrate export.