UniProt functional annotation for Q9NRK6



	UniProt functional annotation for Q9NRK6

UniProt code: Q9NRK6.

Organism: Homo sapiens (Human).

Taxonomy: Eukaryota; Metazoa; Chordata; Craniata; Vertebrata; Euteleostomi; Mammalia; Eutheria; Euarchontoglires; Primates; Haplorrhini; Catarrhini; Hominidae; Homo.

Function: 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). {ECO:0000269|PubMed:22085049, ECO:0000269|PubMed:28315685, ECO:0000269|PubMed:28808058, ECO:0000269|PubMed:33253225}.

Biophysicochemical properties: Kinetic parameters: KM=0.9 uM for Zn (II) mesoporphyrin (ZnMP) {ECO:0000269|PubMed:33253225};

Subunit: 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). {ECO:0000250|UniProtKB:Q9JI39, ECO:0000269|PubMed:23716676, ECO:0000269|PubMed:25063848, ECO:0000269|PubMed:30765471}.

Subcellular location: Mitochondrion inner membrane {ECO:0000250|UniProtKB:Q9JI39}; Multi-pass membrane protein {ECO:0000255}.

Tissue specificity: Ubiquitous. Highly expressed in bone marrow, expressed at intermediate to high levels in skeletal muscle, small intestine, thyroid, heart, brain, placenta, liver, pancreas, prostate, testis, ovary, leukocyte, stomach, spinal cord, lymph node, trachea and adrenal gland, and low levels are found in lung, kidney, spleen, thymus and colon.

Similarity: Belongs to the ABC transporter superfamily. ABCB family. Mitochondrial peptide exporter (TC 3.A.1.212) subfamily. {ECO:0000305}.

Annotations taken from UniProtKB at the EBI.


Organism:		Homo sapiens (Human).
Taxonomy:		Eukaryota; Metazoa; Chordata; Craniata; Vertebrata; Euteleostomi; Mammalia; Eutheria; Euarchontoglires; Primates; Haplorrhini; Catarrhini; Hominidae; Homo.



Function:		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). {ECO:0000269\|PubMed:22085049, ECO:0000269\|PubMed:28315685, ECO:0000269\|PubMed:28808058, ECO:0000269\|PubMed:33253225}.


Biophysicochemical properties:		Kinetic parameters: KM=0.9 uM for Zn (II) mesoporphyrin (ZnMP) {ECO:0000269\|PubMed:33253225};
Subunit:		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). {ECO:0000250\|UniProtKB:Q9JI39, ECO:0000269\|PubMed:23716676, ECO:0000269\|PubMed:25063848, ECO:0000269\|PubMed:30765471}.
Subcellular location:		Mitochondrion inner membrane {ECO:0000250\|UniProtKB:Q9JI39}; Multi-pass membrane protein {ECO:0000255}.
Tissue specificity:		Ubiquitous. Highly expressed in bone marrow, expressed at intermediate to high levels in skeletal muscle, small intestine, thyroid, heart, brain, placenta, liver, pancreas, prostate, testis, ovary, leukocyte, stomach, spinal cord, lymph node, trachea and adrenal gland, and low levels are found in lung, kidney, spleen, thymus and colon.
Similarity:		Belongs to the ABC transporter superfamily. ABCB family. Mitochondrial peptide exporter (TC 3.A.1.212) subfamily. {ECO:0000305}.