ArticleThe Journal of biological chemistry2012
ATP-dependent mitochondrial porphyrin importer ABCB6 protects against phenylhydrazine toxicity.
Article in The Journal of biological chemistry, 2012. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 38 papers.
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
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Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
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Who cites it
38 citing papers in PubMed, 76 citations in OpenAlex.
- The role of ATP-binding Cassette subfamily B member 6 in the inner ear.Nature communications · 2024Article
- Emerging Role of ABC Transporters in Glia Cells in Health and Diseases of the Central Nervous System.Cells · 2024Review
- Targeting ferroptosis in hepatocellular carcinoma.Hepatology international · 2024Review
- Protoporphyrin IX-induced phototoxicity: Mechanisms and therapeutics.Pharmacology & therapeutics · 2023Review
- The human ATP-binding cassette (ABC) transporter superfamily.Human mutation · 2022Review
- Structural Insights into Porphyrin Recognition by the Human ATP-Binding Cassette Transporter ABCB6.Molecules and cells · 2022Article
- ABCB6 polymorphisms are not overly represented in patients with porphyria.Blood advances · 2022Article
- Molecular insights into the human ABCB6 transporter.Cell discovery · 2021Article
- ABCG2 Is Overexpressed on Red Blood Cells in Ph-Negative Myeloproliferative Neoplasms and Potentiates Ruxolitinib-Induced Apoptosis.International journal of molecular sciences · 2021Article
- Insights into the Roles of the Sideroflexins/SLC56 Family in Iron Homeostasis and Iron-Sulfur Biogenesis.Biomedicines · 2021Review
- Cryo-electron microscopy structure of human ABCB6 transporter.Protein science : a publication of the Protein Society · 2020Article
- The ferroptosis and iron-metabolism signature robustly predicts clinical diagnosis, prognosis and immune microenvironment for hepatocellular carcinoma.Cell communication and signaling : CCS · 2020Article
- Review
- 20,000 picometers under the OMM: diving into the vastness of mitochondrial metabolite transport.EMBO reports · 2020Review
- Review
- Investigating host-bacterial interactions among enteric pathogens.BMC genomics · 2019Article
- The human ABCB6 protein is the functional homologue of HMT-1 proteins mediating cadmium detoxification.Cellular and molecular life sciences : CMLS · 2019Article
- Chronic Pressure Overload Results in Deficiency of Mitochondrial Membrane Transporter ABCB7 Which Contributes to Iron Overload, Mitochondrial Dysfunction, Metabolic Shift and Worsens Cardiac Function.Scientific reports · 2019Article
- Regulation of mitochondrial iron homeostasis by sideroflexin 2.The journal of physiological sciences : JPS · 2019Article
- The multifaceted contributions of mitochondria to cellular metabolism.Nature cell biology · 2018Review
Corrections and comments
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Authors and funding
12 authors at 1 institution in 1 country.
Funding
Abstract
Abcb6 is a mammalian mitochondrial ATP-binding cassette (ABC) transporter that regulates de novo porphyrin synthesis. In previous studies, haploinsufficient (Abcb6(+/-)) embryonic stem cells showed impaired porphyrin synthesis. Unexpectedly, Abcb6(-/-) mice derived from these stem cells appeared phenotypically normal. We hypothesized that other ATP-dependent and/or -independent mechanisms conserve porphyrins. Here, we demonstrate that Abcb6(-/-) mice lack mitochondrial ATP-driven import of coproporphyrin III. Gene expression analysis revealed that loss of Abcb6 results in up-regulation of compensatory porphyrin and iron pathways, associated with elevated protoporphyrin IX (PPIX). Phenylhydrazine-induced stress caused higher mortality in Abcb6(-/-) mice, possibly because of sustained elevation of PPIX and an inability to convert PPIX to heme despite elevated ferrochelatase levels. Therefore, Abcb6 is the sole ATP-dependent porphyrin importer, and loss of Abcb6 produces up-regulation of heme and iron pathways necessary for normal development. However, under extreme demand for porphyrins (e.g. phenylhydrazine stress), these adaptations appear inadequate, which suggests that under these conditions Abcb6 is important for optimal survival.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.