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Details on Person UniProt:O75027-3 ABCB7

Class:IdReferenceIsoform:8973151
_chainChangeLogtransit peptide:1-22 added on Fri February 17 2017;chain:23-752 added on Fri February 17 2017
_displayNameUniProt:O75027-3 ABCB7
_timestamp2025-02-21 19:24:49
chaintransit peptide:1-22
chain:23-752
checksumB1FFA57ABD24FB90
commentFUNCTION Exports glutathione-coordinated iron-sulfur clusters such as [2Fe-2S]-(GS)4 cluster from the mitochondria to the cytosol in an ATP-dependent manner allowing the assembly of the cytosolic iron-sulfur (Fe/S) cluster-containing proteins and participates in iron homeostasis (PubMed:10196363, PubMed:17192393, PubMed:33157103). Moreover, through a functional complex formed of ABCB7, FECH and ABCB10, also plays a role in the cellular iron homeostasis, mitochondrial function and heme biosynthesis (PubMed:30765471). In cardiomyocytes, regulates cellular iron homeostasis and cellular reactive oxygen species (ROS) levels through its interaction with COX4I1 (By similarity). May also play a role in hematopoiesis (By similarity).CATALYTIC ACTIVITY (glutathione)4[2Fe(III)-2S] cluster(in) + ATP + H2O = (glutathione)4[2Fe(III)-2S] cluster(out) + ADP + phosphate + H(+)ACTIVITY REGULATION ATPase activity is stimulated by glutathione.BIOPHYSICOCHEMICAL PROPERTIES Homodimer or heterodimer (PubMed:30765471). Interacts with C10orf88/PAAT (PubMed:25063848). Forms a complex with ABCB10 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). Interacts with ATP5F1A (By similarity). Interacts with COX4I1; this interaction allows the regulation of cellular iron homeostasis and cellular reactive oxygen species (ROS) levels in cardiomyocytes (By similarity).INTERACTION The disease is caused by variants affecting the gene represented in this entry.SIMILARITY Belongs to the ABC transporter superfamily. ABCB family. Heavy Metal importer (TC 3.A.1.210) subfamily.ONLINE INFORMATION Database for mutations in ABC proteins
created[InstanceEdit:8964659] Weiser, JD
descriptionrecommendedName: fullName evidence="20"Iron-sulfur clusters transporter ABCB7, mitochondrial alternativeName: fullName evidence="20"ATP-binding cassette sub-family B member 7, mitochondrial alternativeName: ATP-binding cassette transporter 7 shortName: ABC transporter 7 protein
geneNameABCB7
ABC7
identifierO75027
isoformParent
isSequenceChangedFALSE
keyword3D-structure
Acetylation
Alternative splicing
ATP-binding
Disease variant
Membrane
Mitochondrion
Mitochondrion inner membrane
Nucleotide-binding
Phosphoprotein
Proteomics identification
Reference proteome
Transit peptide
Transmembrane
Transmembrane helix
Transport
modified[InstanceEdit:9836292] Weiser, Joel, 2023-05-25
[InstanceEdit:9852000] Weiser, Joel, 2023-11-03
[InstanceEdit:9862192] Weiser, Joel, 2024-02-26
[InstanceEdit:9917590] Weiser, Joel, 2024-08-09
[InstanceEdit:9926675] Weiser, Joel, 2024-11-03
[InstanceEdit:9939033] Weiser, Joel, 2025-02-21
nameABCB7
referenceDatabase[ReferenceDatabase:2] UniProt
referenceGene[ReferenceDNASequence:8962098] ENSEMBL:ENSG00000131269 ABCB7 [Homo sapiens]
secondaryIdentifierABCB7_HUMAN
G3XAC4
O75345
Q5VWY7
Q5VWY8
Q9BRE1
Q9UND1
Q9UP01
sequenceLength752
species[Species:48887] Homo sapiens
variantIdentifierO75027-3
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