ATP-binding cassette sub-family B member 10, mitochondrial

Definition:

Catalyzes the export of an unknown physiological substrate from the mitochondrial matrix to the cytosol in an ATP-dependent manner. 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. 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. In addition may be involved in mitochondrial unfolded protein response (UPRmt) signaling pathway, although ABCB10 probably does not participate in peptide export from mitochondria.

References:

[1]. Masato Yano, et al. ABCB10 depletion reduces unfolded protein response in mitochondria. Biochem Biophys Res Commun. 2017 Apr 29;486(2):465-469. [Content Brief]

[2]. Melissa Martinez, et al. Stimulation of the human mitochondrial transporter ABCB10 by zinc-mesoporphrin. PLoS One. 2020 Nov 30;15(11):e0238754. [Content Brief]

[3]. Leilei Tang, et al. Human mitochondrial ATP-binding cassette transporter ABCB10 is required for efficient red blood cell development. Br J Haematol. 2012 Apr;157(1):151-4. [Content Brief]

[4]. Alexandra Seguin, et al. Reductions in the mitochondrial ABC transporter Abcb10 affect the transcriptional profile of heme biosynthesis genes. J Biol Chem. 2017 Sep 29;292(39):16284-16299. [Content Brief]

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