ArticlePloS one2011
Iron insufficiency compromises motor neurons and their mitochondrial function in Irp2-null mice.
Article in PloS one, 2011. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 47 papers.
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Who cites it
47 citing papers in PubMed, 62 citations in OpenAlex.
- Iron accumulation in deep cortical layers accounts for MRI signal abnormalities in ALS: correlating 7 tesla MRI and pathology.PloS one · 2012Trial
- What causes loss of muscle mass in primary sarcopenia: A MUNIX analysis and correlation with plasma biomarkers.Acta neurologica Belgica · 2026Article
- The Underestimated Role of Iron in Frontotemporal Dementia: A Narrative Review.International journal of molecular sciences · 2024Review
- Iron regulatory protein 2 contributes to antimicrobial immunity by preserving lysosomal function in macrophages.Proceedings of the National Academy of Sciences of the United States of America · 2024Article
- A commentary on studies of brain iron accumulation during ageing.Journal of biological inorganic chemistry : JBIC : a publication of the Society of Biological Inorganic Chemistry · 2024Review
- Ablation of Iron Regulatory Protein 2 produces a neurological disorder characterized by motor, somatosensory, and executive dysfunction in mice.Current research in neurobiology · 2024Article
- Review
- Ferrodifferentiation regulates neurodevelopment via ROS generation.Science China. Life sciences · 2023Article
- A novel cell-free intrathecal approach with PRP for the treatment of spinal cord multiple sclerosis in cats.Inflammation and regeneration · 2022Article
- Neuroprotective Potential of Intranasally Delivered Sulforaphane-Loaded Iron Oxide Nanoparticles Against Cisplatin-Induced Neurotoxicity.Neurotoxicity research · 2022Article
- Iron Homeostasis in the CNS: An Overview of the Pathological Consequences of Iron Metabolism Disruption.International journal of molecular sciences · 2022Review
- Disruption of cellular iron homeostasis byBrain communications · 2022Article
- Mechanisms of cellular iron sensing, regulation of erythropoiesis and mitochondrial iron utilization.Seminars in hematology · 2021Review
- Mammalian mitochondrial iron-sulfur cluster biogenesis and transfer and related human diseases.Biophysics reports · 2021Article
- Protective Effects of Hif2 Inhibitor PT-2385 on a Neurological Disorder Induced by Deficiency of Irp2.Frontiers in neuroscience · 2021Article
- Biomaterials to Neuroprotect the Stroke Brain: A Large Opportunity for Narrow Time Windows.Cells · 2020Review
- Intranasal Delivery of Nanoformulations: A Potential Way of Treatment for Neurological Disorders.Molecules (Basel, Switzerland) · 2020Review
- Irp2 regulates insulin production through iron-mediated Cdkal1-catalyzed tRNA modification.Nature communications · 2020Article
- The Effects of Early-Life Iron Deficiency on Brain Energy Metabolism.Neuroscience insights · 2020Review
- The indispensable role of mammalian iron sulfur proteins in function and regulation of multiple diverse metabolic pathways.Biometals : an international journal on the role of metal ions in biology, biochemistry, and medicine · 2019Review
Corrections and comments
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Authors and funding
10 authors at 5 institutions in 3 countries.
Funding
Abstract
Genetic ablation of Iron Regulatory Protein 2 (Irp2, Ireb2), which post-transcriptionally regulates iron metabolism genes, causes a gait disorder in mice that progresses to hind-limb paralysis. Here we have demonstrated that misregulation of iron metabolism from loss of Irp2 causes lower motor neuronal degeneration with significant spinal cord axonopathy. Mitochondria in the lumbar spinal cord showed significantly decreased Complex I and II activities, and abnormal morphology. Lower motor neurons appeared to be the most adversely affected neurons, and we show that functional iron starvation due to misregulation of iron import and storage proteins, including transferrin receptor 1 and ferritin, may have a causal role in disease. We demonstrated that two therapeutic approaches were beneficial for motor neuron survival. First, we activated a homologous protein, IRP1, by oral Tempol treatment and found that axons were partially spared from degeneration. Secondly, we genetically decreased expression of the iron storage protein, ferritin, to diminish functional iron starvation. These data suggest that functional iron deficiency may constitute a previously unrecognized molecular basis for degeneration of motor neurons in mice.
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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.