ArticleThe Journal of biological chemistry2014
Iron regulatory protein-1 protects against mitoferrin-1-deficient porphyria.
Article in The Journal of biological chemistry, 2014. The graph could read no effect estimate from its abstract, so it casts no vote on the map. An erratum has been issued. Cited by 29 papers.
What it found
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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
29 citing papers in PubMed, 40 citations in OpenAlex.
- Genetic testing to distinguish X-linked protoporphyria from erythropoietic protoporphyria informed iron replacement therapy leading to reversal of severe protoporphyric hepatopathy.Molecular genetics and metabolism reports · 2026Article
- Molecular characterization underlying IFN-α2 treatment in polycythemia vera: a transcriptomic overview.Molecular and cellular biochemistry · 2025Article
- Insights on the crosstalk among different cell death mechanisms.Cell death discovery · 2025Review
- Nanomaterials targeting iron homeostasis: a promising strategy for cancer treatment.Frontiers in bioengineering and biotechnology · 2025Review
- Iron Metabolism in the Disorders of Heme Biosynthesis.Metabolites · 2022Review
- Iron in Porphyrias: Friend or Foe?Diagnostics (Basel, Switzerland) · 2022Review
- Regulation of Heme Synthesis by Mitochondrial Homeostasis Proteins.Frontiers in cell and developmental biology · 2022Review
- The immunometabolite itaconate inhibits heme synthesis and remodels cellular metabolism in erythroid precursors.Blood advances · 2021Article
- Review
- Inflaming the Brain with Iron.Antioxidants (Basel, Switzerland) · 2021Review
- Effects of Iron and Zinc on Mitochondria: Potential Mechanisms of Glaucomatous Injury.Frontiers in cell and developmental biology · 2021Review
- Article
- Mutations in the iron-sulfur cluster biogenesis protein HSCB cause congenital sideroblastic anemia.The Journal of clinical investigation · 2020Article
- Iron-responsive-like elements and neurodegenerative ferroptosis.Learning & memory (Cold Spring Harbor, N.Y.) · 2020Review
- The SLC25 Carrier Family: Important Transport Proteins in Mitochondrial Physiology and Pathology.Physiology (Bethesda, Md.) · 2020Review
- Review
- Review
- Zebrafish as a model system to delineate the role of heme and iron metabolism during erythropoiesis.Molecular genetics and metabolism · 2019Review
- Feasibility of cellular bioenergetics as a biomarker in porphyria patients.Molecular genetics and metabolism reports · 2019Article
- The Role of Iron Regulation in Immunometabolism and Immune-Related Disease.Frontiers in molecular biosciences · 2019Review
Corrections and comments
- Erratum issued
Authors and funding
26 authors at 8 institutions in 2 countries.
Funding
Abstract
Mitochondrial iron is essential for the biosynthesis of heme and iron-sulfur ([Fe-S]) clusters in mammalian cells. In developing erythrocytes, iron is imported into the mitochondria by MFRN1 (mitoferrin-1, SLC25A37). Although loss of MFRN1 in zebrafish and mice leads to profound anemia, mutant animals showed no overt signs of porphyria, suggesting that mitochondrial iron deficiency does not result in an accumulation of protoporphyrins. Here, we developed a gene trap model to provide in vitro and in vivo evidence that iron regulatory protein-1 (IRP1) inhibits protoporphyrin accumulation. Mfrn1(+/gt);Irp1(-/-) erythroid cells exhibit a significant increase in protoporphyrin levels. IRP1 attenuates protoporphyrin biosynthesis by binding to the 5'-iron response element (IRE) of alas2 mRNA, inhibiting its translation. Ectopic expression of alas2 harboring a mutant IRE, preventing IRP1 binding, in Mfrn1(gt/gt) cells mimics Irp1 deficiency. Together, our data support a model whereby impaired mitochondrial [Fe-S] cluster biogenesis in Mfrn1(gt/gt) cells results in elevated IRP1 RNA-binding that attenuates ALAS2 mRNA translation and protoporphyrin accumulation.
Indexed as
Identifiers
What OpenQuestion holds
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.