ABCB10
entrez
23456
canonical
uniprot
Q9NRK6
canonical
ensembl
ENSG00000135776
resolver
ensp
ENSP00000355637
resolver
ensp
ENSP00000716553
resolver
enst
ENST00000344517
resolver
genesymbol
ABCB10
resolver
hgnc
HGNC:41
resolver
uniprot
Q13040
resolver
uniprot
Q6P1Q8
resolver
uniprot
Q9H3V0
resolver
uniprot_entry
ABCBA_HUMAN
resolver
uniprot_sec
Q13040
resolver
uniprot_sec
Q6P1Q8
resolver
uniprot_sec
Q9H3V0
resolver
activity_regulation
ACTIVITY REGULATION: Cholesterol promotes the ATPase activity of ABCB10 in a dose-dependent manner. {ECO:0000269|PubMed:37041204}.
uniprot
proteins
amino_acid_sequence
MRGPPAWPLRLLEPPSPAEPGRLLPVACVWAAASRVPGSLSPFTGLRPARLWGAGPALLWGVGAARRWRSGCRGGGPGASRGVLGLARLLGLWARGPGSCRCGAFAGPGAPRLPRARFPGGPAAAAWAGDEAWRRGPAAPPGDKGRLRPAAAGLPEARKLLGLAYPERRRLAAAVGFLTMSSVISMSAPFFLGKIIDVIYTNPTVDYSDNLTRLCLGLSAVFLCGAAANAIRVYLMQTSGQRIVNRLRTSLFSSILRQEVAFFDKTRTGELINRLSSDTALLGRSVTENLSDGLRAGAQASVGISMMFFVSPNLATFVLSVVPPVSIIAVIYGRYLRKLTKVTQDSLAQATQLAEERIGNVRTVRAFGKEMTEIEKYASKVDHVMQLARKEAFARAGFFGATGLSGNLIVLSVLYKGGLLMGSAHMTVGELSSFLMYAFWVGISIGGLSSFYSELMKGLGAGGRLWELLEREPKLPFNEGVILNEKSFQGALEFKNVHFAYPARPEVPIFQDFSLSIPSGSVTALVGPSGSGKSTVLSLLLRLYDPASGTISLDGHDIRQLNPVWLRSKIGTVSQEPILFSCSIAENIAYGADDPSSVTAEEIQRVAEVANAVAFIRNFPQGFNTVVGEKGVLLSGGQKQRIAIARALLKNPKILLLDEATSALDAENEYLVQEALDRLMDGRTVLVIAHRLSTIKNANMVAVLDQGKITEYGKHEELLSKPNGIYRKLMNKQSFISA
uniprot
proteins
ec_number
7.6.2.-
uniprot
proteins
function
FUNCTION: ATP-dependent transporter located in the mitochondrial inner membrane that catalyzes the export of biliverdin from the mitochondrial matrix, and plays a crucial role in hemoglobin synthesis and antioxidative stress (PubMed:22085049, PubMed:28315685, PubMed:28808058, PubMed:34011630, PubMed:37041204). Participates in the early step of the heme biosynthetic process during insertion of iron into protoporphyrin IX (PPIX) (PubMed:22085049, PubMed:28808058). Involved in the stabilization of the iron transporter mitoferrin-1/SLC25A37 (By similarity). 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:0000250|UniProtKB:Q9JI39, ECO:0000269|PubMed:22085049, ECO:0000269|PubMed:28315685, ECO:0000269|PubMed:28808058, ECO:0000269|PubMed:34011630, ECO:0000269|PubMed:37041204}.
uniprot
proteins
mass_dalton
79148
uniprot
proteins
mutagenesis
MUTAGEN 215; /note="C->S: Does not affect ATPase activity; when associated with L-224 and G-582. Activated by Zn (II) mesoporphyrin; when associated with L-224 and G-582."; /evidence="ECO:0000269|PubMed:33253225"; MUTAGEN 224; /note="C->L: Does not affect ATPase activity; when associated with S-215 and G-582. Activated by Zn (II) mesoporphyrin; when associated with S-215 and G-582."; /evidence="ECO:0000269|PubMed:33253225"; MUTAGEN 229; /note="N->A: Does not affect ATPase activity in the absence or presence of biliverdin. No increased ATPase activity in the presence of biliverdin; when associated with A-398. Increased ATPase activity in the absence of biliverdin compared to WT; when associated with A-407. No increased ATPase activity in the presence of biliverdin; when associated with A-407. No increased ATPase activity in the presence of biliverdin; when associated with A-398 and A-407."; /evidence="ECO:0000269|PubMed:37041204"; MUTAGEN 398; /note="F->A: No increased ATPase activity in the presence of biliverdin. No increased ATPase activity in the presence of biliverdin; when associated with A-229. No increased ATPase activity in the presence of biliverdin; when associated with A-407. No increased ATPase activity in the presence of biliverdin; when associated with A-229 and A-407."; /evidence="ECO:0000269|PubMed:37041204"; MUTAGEN 407; /note="N->A: Increased ATPase activity in the absence of biliverdin compared to WT. No increased ATPase activity in the presence of biliverdin. Increased ATPase activity in the absence of biliverdin compared to WT; when associated with A-229. No increased ATPase activity in the presence of biliverdin; when associated with A-229. No increased ATPase activity in the presence of biliverdin; when associated with A-398. No increased ATPase activity in the presence of biliverdin; when associated with A-229 and A-398."; /evidence="ECO:0000269|PubMed:37041204"; MUTAGEN 533; /note="K->E: Increases hemoglobin biosynthetic process."; /evidence="ECO:0000269|PubMed:28808058"; MUTAGEN 582; /note="C->G: Does not affect ATPase activity; when associated with S-215 and L-224. Activated by Zn (II) mesoporphyrin; when associated with S-215 and L-224."; /evidence="ECO:0000269|PubMed:33253225"; MUTAGEN 635; /note="S->R: Does not rescue hemoglobin and heme biosynthetic process."; /evidence="ECO:0000269|PubMed:28808058"; MUTAGEN 638; /note="Q->H: Does not rescue hemoglobin and heme biosynthetic process."; /evidence="ECO:0000269|PubMed:28808058"; MUTAGEN 658; /note="D->A: Does not rescue hemoglobin and heme biosynthetic process."; /evidence="ECO:0000269|PubMed:28808058"; MUTAGEN 659; /note="E->A: Does not rescue hemoglobin and heme biosynthetic process."; /evidence="ECO:0000269|PubMed:28808058
uniprot
proteins
protein_family
ABC transporter superfamily
uniprot
proteins
protein_family
ABCB family
uniprot
proteins
protein_family
Mitochondrial peptide exporter (TC 3.A.1.212) subfamily
uniprot
proteins
pubmed
10922475
uniprot
proteins
pubmed
11829140
uniprot
proteins
pubmed
15489334
uniprot
proteins
pubmed
16710414
uniprot
proteins
pubmed
16959974
uniprot
proteins
pubmed
19608861
uniprot
proteins
pubmed
21269460
uniprot
proteins
pubmed
22085049
uniprot
proteins
pubmed
23716676
uniprot
proteins
pubmed
24275569
uniprot
proteins
pubmed
25063848
uniprot
proteins
pubmed
25944712
uniprot
proteins
pubmed
28315685
uniprot
proteins
pubmed
28808058
uniprot
proteins
pubmed
30765471
uniprot
proteins
pubmed
33253225
uniprot
proteins
pubmed
34011630
uniprot
proteins
pubmed
37041204
uniprot
proteins
pubmed
40105103
uniprot
proteins
pubmed
7766993
uniprot
proteins
sequence_length
738
uniprot
proteins
subcellular_location
SUBCELLULAR LOCATION: Mitochondrion inner membrane {ECO:0000250|UniProtKB:Q9JI39}; Multi-pass membrane protein {ECO:0000269|PubMed:23716676}.
uniprot
proteins
transmembrane_region
TRANSMEM 169..192; /note="Helical"; /evidence="ECO:0000269|PubMed:23716676, ECO:0007744|PDB:4AYT"; TRANSMEM 214..236; /note="Helical"; /evidence="ECO:0000269|PubMed:23716676, ECO:0007744|PDB:4AYT"; TRANSMEM 288..310; /note="Helical"; /evidence="ECO:0000269|PubMed:23716676, ECO:0007744|PDB:4AYT"; TRANSMEM 314..333; /note="Helical"; /evidence="ECO:0000269|PubMed:23716676, ECO:0007744|PDB:4AYT"; TRANSMEM 393..416; /note="Helical"; /evidence="ECO:0000269|PubMed:23716676, ECO:0007744|PDB:4AYT"; TRANSMEM 431..452; /note="Helical"; /evidence="ECO:0000269|PubMed:23716676, ECO:0007744|PDB:4AYT
uniprot
proteins