PMID-sentid Pub_year Sent_text comp_official_name comp_offsetprotein_name organism prot_offset 35041979-1 2022 ATP-binding cassette subfamily B member 7 (ABCB7) is localized in the inner membrane of mitochondria, playing a critical role in iron metabolism. Iron 129-133 ATP binding cassette subfamily B member 7 Homo sapiens 0-41 35041979-1 2022 ATP-binding cassette subfamily B member 7 (ABCB7) is localized in the inner membrane of mitochondria, playing a critical role in iron metabolism. Iron 129-133 ATP binding cassette subfamily B member 7 Homo sapiens 43-48 32337520-4 2020 The mitochondrial inner membrane protein Atm1p can transport glutathione-coordinated iron-sulfur clusters, which may connect the mitochondrial and cytosolic iron-sulfur cluster assembly systems. Iron 85-89 ATP binding cassette subfamily B member 7 Homo sapiens 41-46 33689857-2 2021 ABCB7 also plays a central role in the maturation of cytosolic iron-sulfur (Fe/S) cluster-containing proteins, and mutations can cause a series of mitochondrial defects. Iron 76-78 ATP binding cassette subfamily B member 7 Homo sapiens 0-5 33157103-2 2021 Iron-sulfur cofactors are assembled primarily in mitochondria and are then exported to the cytosol by use of an ABCB7 transporter. Iron 0-4 ATP binding cassette subfamily B member 7 Homo sapiens 112-117 20481466-5 2010 Fe-S cluster biogenesis takes place essentially in every tissue of humans, and products of human disease genes, including frataxin, GLRX5, ISCU, and ABCB7, have important roles in the process. Iron 0-4 ATP binding cassette subfamily B member 7 Homo sapiens 149-154 31772327-4 2020 Here, we show that ABC transporter subfamily B member 7 (ABCB7), one of the mitochondrial iron transporters, induces the hypoxia-independent accumulation of hypoxia-inducible factor 1 alpha by controlling intracellular iron homeostasis and inhibits both apoptotic and non-apoptotic cell death. Iron 90-94 ATP binding cassette subfamily B member 7 Homo sapiens 57-62 31772327-4 2020 Here, we show that ABC transporter subfamily B member 7 (ABCB7), one of the mitochondrial iron transporters, induces the hypoxia-independent accumulation of hypoxia-inducible factor 1 alpha by controlling intracellular iron homeostasis and inhibits both apoptotic and non-apoptotic cell death. Iron 219-223 ATP binding cassette subfamily B member 7 Homo sapiens 57-62 31772327-7 2020 Therefore, our results support that ABCB7 is crucial in controlling both apoptotic and non-apoptotic cell death and indicate that the fine-tuning of intracellular iron homeostasis may be a novel anticancer strategy. Iron 163-167 ATP binding cassette subfamily B member 7 Homo sapiens 36-41 27211273-7 2016 We describe cryptic splicing events in the HSCs of SF3B1-mutant MDS, and our data support a model in which NMD-induced downregulation of the iron exporter ABCB7 mRNA transcript resulting from aberrant splicing caused by mutant SF3B1 underlies the increased mitochondrial iron accumulation found in MDS patients with RS. Iron 141-145 ATP binding cassette subfamily B member 7 Homo sapiens 155-160 27211273-7 2016 We describe cryptic splicing events in the HSCs of SF3B1-mutant MDS, and our data support a model in which NMD-induced downregulation of the iron exporter ABCB7 mRNA transcript resulting from aberrant splicing caused by mutant SF3B1 underlies the increased mitochondrial iron accumulation found in MDS patients with RS. Iron 271-275 ATP binding cassette subfamily B member 7 Homo sapiens 155-160 27590019-2 2016 This complex demonstrates physiologically relevant solution chemistry and is a viable substrate for iron-sulfur cluster transport by Atm1p exporter protein. Iron 100-104 ATP binding cassette subfamily B member 7 Homo sapiens 133-138 26728034-1 2016 BACKGROUND: Mitochondria play essential biological functions including the synthesis and trafficking of porphyrins and iron/sulfur clusters (ISC), processes that in mammals involve the mitochondrial ATP-Binding Cassette (ABC) transporters ABCB6 and ABCB7, respectively. Iron 119-123 ATP binding cassette subfamily B member 7 Homo sapiens 249-254 26517908-4 2015 In particular, ABC subfamily B member 7 (ABCB7) and its homologues in yeast and plants are required for iron-sulfur (Fe-S) cluster biosynthesis outside of the mitochondria, whereas ABCB10 is involved in haem biosynthesis. Iron 117-121 ATP binding cassette subfamily B member 7 Homo sapiens 41-46 24584132-0 2014 A structural model for glutathione-complexed iron-sulfur cluster as a substrate for ABCB7-type transporters. Iron 45-49 ATP binding cassette subfamily B member 7 Homo sapiens 84-89 21926174-0 2011 Leucine biosynthesis regulates cytoplasmic iron-sulfur enzyme biogenesis in an Atm1p-independent manner. Iron 43-47 ATP binding cassette subfamily B member 7 Homo sapiens 79-84 30765471-4 2019 In erythroid cells, loss of ABCB7 altered cellular iron distribution and caused mitochondrial iron overload due to activation of iron regulatory proteins 1 and 2 in the cytosol and to upregulation of the mitochondrial iron importer, mitoferrin-1. Iron 51-55 ATP binding cassette subfamily B member 7 Homo sapiens 28-33 30765471-4 2019 In erythroid cells, loss of ABCB7 altered cellular iron distribution and caused mitochondrial iron overload due to activation of iron regulatory proteins 1 and 2 in the cytosol and to upregulation of the mitochondrial iron importer, mitoferrin-1. Iron 94-98 ATP binding cassette subfamily B member 7 Homo sapiens 28-33 30765471-4 2019 In erythroid cells, loss of ABCB7 altered cellular iron distribution and caused mitochondrial iron overload due to activation of iron regulatory proteins 1 and 2 in the cytosol and to upregulation of the mitochondrial iron importer, mitoferrin-1. Iron 94-98 ATP binding cassette subfamily B member 7 Homo sapiens 28-33 30765471-6 2019 In ABCB7-depleted cells, defective heme biosynthesis resulted from translational repression of ALAS2 by iron regulatory proteins and from decreased stability of the terminal enzyme ferrochelatase. Iron 104-108 ATP binding cassette subfamily B member 7 Homo sapiens 3-8 30765471-9 2019 Our studies not only underscore the importance of ABCB7 for mitochondrial Fe-S biogenesis and iron homeostasis, but also provide the biochemical characterization of a multiprotein complex required for heme biosynthesis. Iron 94-98 ATP binding cassette subfamily B member 7 Homo sapiens 50-55 25556595-1 2015 The glutathione-coordinated [2Fe-2S] cluster is demonstrated to be a viable and likely substrate for physiological iron-sulfur cluster transport by Atm1p, a mitochondrial ABC export protein. Iron 115-119 ATP binding cassette subfamily B member 7 Homo sapiens 148-153 21380928-0 2011 Loss of ABCB7 gene: pathogenesis of mitochondrial iron accumulation in erythroblasts in refractory anemia with ringed sideroblast with isodicentric (X)(q13). Iron 50-54 ATP binding cassette subfamily B member 7 Homo sapiens 8-13 21380928-4 2011 We identify the loss of the ABCB7 (ATP-binding cassette subfamily B member-7) gene, which is located on Xq13 and is involved in mitochondrial iron transport to the cytosol, by fluorescent in situ hybridization (FISH) analysis and the decreased expression level of ABCB7 mRNA in the patient"s bone marrow cells. Iron 142-146 ATP binding cassette subfamily B member 7 Homo sapiens 28-33 21380928-4 2011 We identify the loss of the ABCB7 (ATP-binding cassette subfamily B member-7) gene, which is located on Xq13 and is involved in mitochondrial iron transport to the cytosol, by fluorescent in situ hybridization (FISH) analysis and the decreased expression level of ABCB7 mRNA in the patient"s bone marrow cells. Iron 142-146 ATP binding cassette subfamily B member 7 Homo sapiens 264-269 21380928-7 2011 These findings suggest that loss of the ABCB7 gene may be a pathogenetic factor underlying mitochondrial iron accumulation in RARS patients with idicXq13. Iron 105-109 ATP binding cassette subfamily B member 7 Homo sapiens 40-45 19453295-11 2009 RNA interference of the putative Drosophila orthologue of human ABCB7, a mitochondrial transporter involved in cytoplasmic ISC protein maturation, restored Fer1HCH transcript levels of iron-treated mbn-dmfrn cells to those of control cells grown in normal medium. Iron 185-189 ATP binding cassette subfamily B member 7 Homo sapiens 64-69 20408843-6 2010 ABCB7, transporting iron from mitochondria to cytosol and associated with inherited ring sideroblast formation was severely suppressed and expression decreased with differentiation, while increasing in NBM cultures. Iron 20-24 ATP binding cassette subfamily B member 7 Homo sapiens 0-5 20393584-2 2010 Iron concentrations can rise to toxic levels in mitochondria of excitable cells, often leaving the cytosol iron-depleted, in some forms of neurodegeneration with brain accumulation (NBIA) or following mutations in genes associated with mitochondrial functions, such as ABCB7 in X-linked sideroblastic anemia with ataxia (XLSA/A) or the genes encoding frataxin in Friedreich"s ataxia (FRDA). Iron 0-4 ATP binding cassette subfamily B member 7 Homo sapiens 269-274 19786205-4 2009 The identification of other genes, such as adenosine triphosphate (ATP) binding cassette B7 (ABCB7) and glutaredoxin 5 (GLRX5), has strengthened the role of iron sulfur cluster biogenesis in sideroblast formation and revealed a complex interplay between pathways of mitochondrial iron utilization and cytosolic iron sensing by the iron-regulatory proteins (IRPs). Iron 157-161 ATP binding cassette subfamily B member 7 Homo sapiens 93-98 19786205-4 2009 The identification of other genes, such as adenosine triphosphate (ATP) binding cassette B7 (ABCB7) and glutaredoxin 5 (GLRX5), has strengthened the role of iron sulfur cluster biogenesis in sideroblast formation and revealed a complex interplay between pathways of mitochondrial iron utilization and cytosolic iron sensing by the iron-regulatory proteins (IRPs). Iron 280-284 ATP binding cassette subfamily B member 7 Homo sapiens 93-98 19786205-4 2009 The identification of other genes, such as adenosine triphosphate (ATP) binding cassette B7 (ABCB7) and glutaredoxin 5 (GLRX5), has strengthened the role of iron sulfur cluster biogenesis in sideroblast formation and revealed a complex interplay between pathways of mitochondrial iron utilization and cytosolic iron sensing by the iron-regulatory proteins (IRPs). Iron 280-284 ATP binding cassette subfamily B member 7 Homo sapiens 93-98 18398482-4 2008 XLSA/A is caused by partial inactivating mutations of the ABCB7 ATP-binding cassette transporter gene, which functions to enable transport of iron from the mitochondria to the cytoplasm. Iron 142-146 ATP binding cassette subfamily B member 7 Homo sapiens 58-63 18637800-5 2008 New forms have been recognized as being linked to the deficient function of mitochondrial proteins involved in iron-sulphur cluster biogenesis, such as ABCB7 and GLRX5, which are extremely rare but represent important biological models. Iron 111-115 ATP binding cassette subfamily B member 7 Homo sapiens 152-157 18398482-5 2008 Furthermore, ABCB7 gene silencing in HeLa cells causes an accumulation of iron in the mitochondria. Iron 74-78 ATP binding cassette subfamily B member 7 Homo sapiens 13-18 17656326-6 2007 The yeast protein Atm1p plays a critical role in the transport of Fe/S clusters to the cytosol, and a similar function has been attributed to the homologous human proteins MTABC3 and ABC7. Iron 66-68 ATP binding cassette subfamily B member 7 Homo sapiens 183-187 17726528-4 2007 The ATP-binding cassette (ABC) transporters ABCB6 and ABCB7 are also involved in iron homeostasis. Iron 81-85 ATP binding cassette subfamily B member 7 Homo sapiens 54-59 17192393-0 2007 RNA silencing of the mitochondrial ABCB7 transporter in HeLa cells causes an iron-deficient phenotype with mitochondrial iron overload. Iron 77-81 ATP binding cassette subfamily B member 7 Homo sapiens 35-40 17192393-1 2007 X-linked sideroblastic anemia with ataxia (XLSA/A) is caused by defects of the transporter ABCB7 and is characterized by mitochondrial iron deposition and excess of protoporphyrin in erythroid cells. Iron 135-139 ATP binding cassette subfamily B member 7 Homo sapiens 91-96 17192393-3 2007 The phenotype of the ABCB7-deficient cells was characterized by a strong reduction in proliferation rate that was not rescued by iron supplementation, by evident signs of iron deficiency, and by a large approximately 6-fold increase of iron accumulation in the mitochondria that was poorly available to mitochondrial ferritin. Iron 129-133 ATP binding cassette subfamily B member 7 Homo sapiens 21-26 17192393-3 2007 The phenotype of the ABCB7-deficient cells was characterized by a strong reduction in proliferation rate that was not rescued by iron supplementation, by evident signs of iron deficiency, and by a large approximately 6-fold increase of iron accumulation in the mitochondria that was poorly available to mitochondrial ferritin. Iron 171-175 ATP binding cassette subfamily B member 7 Homo sapiens 21-26 17192393-6 2007 The results support the hypothesis that ABCB7 is involved in the transfer of iron from mitochondria to cytosol, and in the maturation of cytosolic Fe/S enzymes. Iron 77-81 ATP binding cassette subfamily B member 7 Homo sapiens 40-45 16467350-2 2006 The mitochondrial ABC half-transporter Abcb7, which is mutated in X-linked sideroblastic anemia with ataxia in humans, is a functional ortholog of yeast Atm1p and is predicted to export a mitochondrially derived metabolite required for cytosolic Fe-S cluster assembly. Iron 246-250 ATP binding cassette subfamily B member 7 Homo sapiens 39-44 16892088-4 2006 Mutations in the adenosine triphosphate-binding cassette protein ABCB7, identified in XLSA with ataxia (XLSA-A), disrupt the maturation of cytosolic (Fe-S) clusters, leading to mitochondrial Fe accumulation. Iron 150-152 ATP binding cassette subfamily B member 7 Homo sapiens 65-70 10196363-3 1999 This gene, ABC7, is an ortholog of the yeast ATM1 gene whose product localizes to the mitochondrial inner membrane and is involved in iron homeostasis. Iron 134-138 ATP binding cassette subfamily B member 7 Homo sapiens 11-15 15528311-5 2005 For instance, the disease X-linked sideroblastic anemia with ataxia (XLSA/A) is due to a mutation in the ATP-binding cassette protein B7 (ABCB7) transporter that is thought to transfer [Fe-S] clusters from the mitochondrion to the cytoplasm. Iron 186-190 ATP binding cassette subfamily B member 7 Homo sapiens 105-136 15528311-5 2005 For instance, the disease X-linked sideroblastic anemia with ataxia (XLSA/A) is due to a mutation in the ATP-binding cassette protein B7 (ABCB7) transporter that is thought to transfer [Fe-S] clusters from the mitochondrion to the cytoplasm. Iron 186-190 ATP binding cassette subfamily B member 7 Homo sapiens 138-143 11050011-0 2000 Human ABC7 transporter: gene structure and mutation causing X-linked sideroblastic anemia with ataxia with disruption of cytosolic iron-sulfur protein maturation. Iron 131-135 ATP binding cassette subfamily B member 7 Homo sapiens 6-10 11050011-1 2000 The human protein ABC7 belongs to the adenosine triphosphate-binding cassette transporter superfamily, and its yeast orthologue, Atm1p, plays a central role in the maturation of cytosolic iron-sulfur (Fe/S) cluster-containing proteins. Iron 201-203 ATP binding cassette subfamily B member 7 Homo sapiens 18-22 12480705-1 2003 A mitochondrial half-type ATP-binding cassette (ABC) protein, ABC7, plays a role in iron homeostasis in mitochondria, and defects in human ABC7 were shown to be responsible for the inherited disease X-linked sideroblastic anemia/ataxia. Iron 84-88 ATP binding cassette subfamily B member 7 Homo sapiens 62-66 12480705-1 2003 A mitochondrial half-type ATP-binding cassette (ABC) protein, ABC7, plays a role in iron homeostasis in mitochondria, and defects in human ABC7 were shown to be responsible for the inherited disease X-linked sideroblastic anemia/ataxia. Iron 84-88 ATP binding cassette subfamily B member 7 Homo sapiens 139-143 12243984-6 2002 The study of X-linked sideroblastic anaemia has shown that the entry of iron into the mitochondria is poorly controlled and able to occur when protoporphyrin production is reduced, as is seen with the ALA-S2 mutations, or when it is increased as has been seen with ABC7 transporter mutations. Iron 72-76 ATP binding cassette subfamily B member 7 Homo sapiens 265-269 11843825-5 2001 ABC7 encodes a mitochondrial half-type ATP Binding Cassette transporter involved in iron homeostasis. Iron 84-88 ATP binding cassette subfamily B member 7 Homo sapiens 0-4 10595578-8 1999 This involves the export of sulfur and possibly iron from mitochondria to the cytosol, a reaction performed by the ABC transporter Atm1p of the mitochondrial inner membrane. Iron 48-52 ATP binding cassette subfamily B member 7 Homo sapiens 131-136 9428742-0 1997 The ABC transporter Atm1p is required for mitochondrial iron homeostasis. Iron 56-60 ATP binding cassette subfamily B member 7 Homo sapiens 20-25 9428742-5 1997 Mitochondria deficient in Atm1p accumulate 30-fold higher levels of free iron as compared to wild-type organelles, i.e. three-fold more than mitochondria deficient in frataxin, the protein mutated in Friedreich"s ataxia. Iron 73-77 ATP binding cassette subfamily B member 7 Homo sapiens 26-31 9428742-6 1997 The increased mitochondrial iron content may be causative of the oxidative damage of heme-containing proteins in delta atm1 cells. Iron 28-32 ATP binding cassette subfamily B member 7 Homo sapiens 119-123 9428742-7 1997 Our data assign an important function to Atm1p in mitochondrial iron homeostasis. Iron 64-68 ATP binding cassette subfamily B member 7 Homo sapiens 41-46 34861039-7 2022 Our study demonstrates that coordinated mis-splicing of mitochondrial transporters TMEM14C and ABCB7 by mutant SF3B1 sequesters iron in mitochondria, causing ring sideroblast formation. Iron 128-132 ATP binding cassette subfamily B member 7 Homo sapiens 95-100 33770183-4 2021 No longer needed for the role of cellular detoxification, ABCB7 appears to be used to transport glutathione-coordinated iron-sulfur clusters from mitochondria to the cytosol. Iron 120-124 ATP binding cassette subfamily B member 7 Homo sapiens 58-63 8175108-4 1994 The highest correlation was found between S-ferritin and the product of chemical iron x ASAT (r = 0.61, p = 0.0001) (log vs. log values). Iron 81-85 ATP binding cassette subfamily B member 7 Homo sapiens 88-92