FEATURES

FAMILY

DESCRIPTION

Also known as ABCBA_HUMAN, ABCB10. Catalyzes the export of an unknown physiological substrate from the mitochondrial matrix to the cytosol in an ATP-dependent manner (PubMed:33253225). May also transport the heme analog Zn (II) mesoporphyrin (ZnMP) in an ATP dependent manner but can't export the heme precursor 5-aminolevulinic acid (ALA) from mitochondria (PubMed:33253225). Plays a role in the early step of the heme biosynthetic process during insertion of iron into protoporphyrin IX (PPIX). In turn participates in hemoglobin synthesis and also protects against oxidative stress (PubMed:28808058, PubMed:22085049). In addition may be involved in mitochondrial unfolded protein response (UPRmt) signaling pathway, although ABCB10 probably does not participate in peptide export from mitochondria (PubMed:28315685). Homodimer or homooligomer (PubMed:23716676, PubMed:30765471). Interacts with PAAT (PubMed:25063848). Interacts with SLC25A37; this interaction stabilizes SLC25A37 and enhances the function of SLC25A37 to import mitochondrial iron during erythroid differentiation (By similarity). Interacts with FECH; this interaction may allow the formation of an oligomeric complex with SLC25A37 (By similarity). Forms a complex with ABCB7 and FECH, where a dimeric FECH bridges ABCB7 and ABCB10 homodimers; this complex may be required for cellular iron homeostasis, mitochondrial function and heme biosynthesis (PubMed:30765471). Interacts with FECH (PubMed:30765471).

EXTERNAL LINKS

ABCB10

Mutations

Mutation(s) distribution

Mutations table