Protein: Iron-sulfur clusters transporter ABCB7, mitochondrial (ATP-binding cassette sub-family B member 7, mitochondrial) (ATP-binding cassette transporter 7) (ABC transporter 7 protein) (Q61102)
Organism: Mus musculus (Mouse)
Function: 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. 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. 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.
Subcellular location: Mitochondrion inner membrane {ECO:0000250|UniProtKB:P40416}; Multi-pass membrane protein {ECO:0000250|UniProtKB:P40416}.
Sequence: MALLAIHSWRWAAAAVAFEKHKHSAVLTRALVSMCGSGPRWSSSQRGASGSARLSQTTESLRNTTQQRWGKDNSRQLLDATKALQTWPLIEKRTCWHGHAGGGLHTDPKEGLKDVDTRKIIKAMLSYVWPEDRPDLRARVAISLGFLGGAKAMNIVVPFMFKYAVDSLNQMSGNMLNLSDAPNTVATMATAVLIGYGVSRAGAAFFNEVRNAVFGKVAQNSIRRIAKNVFLHLHNLDLGFHLSRQTGALSKAIDRGTRGISFVLSALVFNLLPIVFEMMLVSSVLYYKCGAQFALVTLGTLGAYTAFTVAVTRWRTRFRIEMNKADNDAGNAAIDSLLNYETVKYFNNEKYEAQRYDGFLKTYETASLKSTSTLAMLNFGQNAIFSVGLTAIMVLASQGIVAGALTVGDLVMVNGLLFQLSLPLNFLGTVYRETRQALIDMNTLFTLLKVDTRIKDKVMAPPLQITPQTATVAFDNVHFEYIEGQKVLNGVSFEVPAGKKVAIVGGSGSGKSTIVRLLFRFYEPQKGSIYLAGQNLQDVSLESLRRAVGVVPQDAVLFHNTIYYNLLYGNINASPEEVYAVAKLAGLHDAILRMPHGYDTQVGERGLKLSGGEKQRVAIARAILKNPPVILYDEATSSLDSITEETILGAMRDVVKHRTSIFIAHRLSTVVDADEIIVLSQGKVAERGTHYGLLANSSSIYSEMWHTQSNRVQNQDSLGWDAKKESLSKEEERKKLQEEIVNSVKGCGNCSC
InterPro domains: - IPR017871: ABC transporter-like, conserved site (conserved_site) [609-623]
- IPR036640: ABC transporter type 1, transmembrane domain superfamily (homologous_superfamily) [109-457]
- IPR003593: AAA+ ATPase domain (domain) [497-683]
- IPR027417: P-loop containing nucleoside triphosphate hydrolase (homologous_superfamily) [464-725]
- IPR039421: Type 1 protein exporter (family) [80-704]
- IPR003439: ABC transporter-like, ATP-binding domain (domain) [472-706]
- IPR011527: ABC transporter type 1, transmembrane domain (domain) [141-436]
Interaction partners: - Ferrochelatase, mitochondrial
- Mitoferrin-1
- Glutaredoxin-related protein 5, mitochondrial
- Cysteine desulfurase
- 5-aminolevulinate synthase, erythroid-specific, mitochondrial
- Transmembrane protein 14C
- Iron-sulfur cluster assembly enzyme ISCU
- Frataxin, mitochondrial
- Iron-sulfur cluster assembly factor IBA57, mitochondrial
- LYR motif-containing protein 4
Gene Ontology annotations:
Molecular Function (MF): GO:0003674 (molecular function), GO:0005488 (binding), GO:0005515 (protein binding)
Biological Process (BP): GO:0008150 (biological process), GO:0008152 (metabolic process), GO:0065007 (biological regulation), GO:0051179 (localization), GO:0048518 (positive regulation of biological process), GO:0050789 (regulation of biological process), GO:0009987 (cellular process), GO:0050793 (regulation of developmental process), GO:0009058 (biosynthetic process), GO:0044237 (cellular metabolic process), GO:0042440 (pigment metabolic process), GO:0051240 (positive regulation of multicellular organismal process), GO:0006807 (nitrogen compound metabolic process), GO:0050794 (regulation of cellular process), GO:0051234 (establishment of localization), GO:0051239 (regulation of multicellular organismal process), GO:0071704 (organic substance metabolic process), GO:0051094 (positive regulation of developmental process), GO:0048522 (positive regulation of cellular process), GO:0045597 (positive regulation of cell differentiation), GO:2000026 (regulation of multicellular organismal development), GO:1901360 (organic cyclic compound metabolic process), GO:1901564 (organonitrogen compound metabolic process), GO:1901576 (organic substance biosynthetic process), GO:0046483 (heterocycle metabolic process), GO:0006810 (transport), GO:0046148 (pigment biosynthetic process), GO:0045595 (regulation of cell differentiation), GO:0044249 (cellular biosynthetic process), GO:0006725 (cellular aromatic compound metabolic process), GO:0034641 (cellular nitrogen compound metabolic process), GO:0042168 (heme metabolic process), GO:1901566 (organonitrogen compound biosynthetic process), GO:1901362 (organic cyclic compound biosynthetic process), GO:0019438 (aromatic compound biosynthetic process), GO:0018130 (heterocycle biosynthetic process), GO:0060284 (regulation of cell development), GO:0044271 (cellular nitrogen compound biosynthetic process), GO:0033013 (tetrapyrrole metabolic process), GO:0006811 (monoatomic ion transport), GO:0006783 (heme biosynthetic process), GO:0006778 (porphyrin-containing compound metabolic process), GO:0033014 (tetrapyrrole biosynthetic process), GO:0006812 (monoatomic cation transport), GO:0050767 (regulation of neurogenesis), GO:0030001 (metal ion transport), GO:0000041 (transition metal ion transport), GO:0006826 (iron ion transport), GO:1901678 (iron coordination entity transport), GO:0015891 (siderophore transport)
Cellular Component (CC): GO:0005575 (cellular component), GO:0110165 (cellular anatomical entity), GO:0005622 (intracellular anatomical structure), GO:0043226 (organelle), GO:0031975 (envelope), GO:0016020 (membrane), GO:0005737 (cytoplasm), GO:0031090 (organelle membrane), GO:0031967 (organelle envelope), GO:0005739 (mitochondrion), GO:0043229 (intracellular organelle), GO:0043227 (membrane-bounded organelle), GO:0005740 (mitochondrial envelope), GO:0019866 (organelle inner membrane), GO:0043231 (intracellular membrane-bounded organelle), GO:0031966 (mitochondrial membrane), GO:0005743 (mitochondrial inner membrane)