ArticleJournal of neurochemistry2015
Cp/Heph mutant mice have iron-induced neurodegeneration diminished by deferiprone.
Article in Journal of neurochemistry, 2015. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 22 papers.
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Who cites it
22 citing papers in PubMed, 36 citations in OpenAlex.
- How does iron cross the abluminal membrane of the blood-brain barrier.Experimental & molecular medicine · 2026Review
- Metal ions in aging and ocular diseases: biology, pathophysiology, and therapeutic strategies.npj aging · 2026Review
- Increased Complement C4 in a Sparse Neuronal Subset Induces Network-Wide Transcriptomic Alterations in the Prefrontal Cortex.bioRxiv : the preprint server for biology · 2025Article
- The biology of mammalian multi-copper ferroxidases.Biometals : an international journal on the role of metal ions in biology, biochemistry, and medicine · 2023Review
- Ceruloplasmin Deficiency Impaired Brain Iron Metabolism and Behavior in Mice.Cell biochemistry and biophysics · 2022Article
- Interleukin-6 triggers toxic neuronal iron sequestration in response to pathological α-synuclein.Cell reports · 2022Article
- Review
- Article
- Iron Homeostasis and Metabolism: Two Sides of a Coin.Advances in experimental medicine and biology · 2021Review
- Retinal Degeneration and Alzheimer's Disease: An Evolving Link.International journal of molecular sciences · 2020Review
- Oral administration of the iron chelator deferiprone protects against loss of retinal ganglion cells in a mouse model of glaucoma.Experimental eye research · 2020Article
- High Dietary Iron Disrupts Iron Homeostasis and Induces Amyloid-β and Phospho-τ Expression in the Hippocampus of Adult Wild-Type and APP/PS1 Transgenic Mice.The Journal of nutrition · 2019Article
- Dexras1 Deletion and Iron Chelation Promote Neuroprotection in Experimental Optic Neuritis.Scientific reports · 2019Article
- Brain Ceruloplasmin Expression After Experimental Intracerebral Hemorrhage and Protection Against Iron-Induced Brain Injury.Translational stroke research · 2019Article
- Ceruloplasmin and hephaestin jointly protect the exocrine pancreas against oxidative damage by facilitating iron efflux.Redox biology · 2018Article
- Haemochromatosis.Nature reviews. Disease primers · 2018Review
- Severe Iron Metabolism Defects in Mice With Double Knockout of the Multicopper Ferroxidases Hephaestin and Ceruloplasmin.Cellular and molecular gastroenterology and hepatology · 2018Article
- Animal Models of Tremor: Relevance to Human Tremor Disorders.Tremor and other hyperkinetic movements (New York, N.Y.) · 2018Review
- Cerebrospinal fluid cell-free mitochondrial DNA is associated with HIV replication, iron transport, and mild HIV-associated neurocognitive impairment.Journal of neuroinflammation · 2017Observational
- Loss of NCB5OR in the cerebellum disturbs iron pathways, potentiates behavioral abnormalities, and exacerbates harmaline-induced tremor in mice.Metabolic brain disease · 2016Article
Corrections and comments
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Authors and funding
18 authors at 11 institutions in 3 countries.
Funding
Abstract
Brain iron accumulates in several neurodegenerative diseases and can cause oxidative damage, but mechanisms of brain iron homeostasis are incompletely understood. Patients with mutations in the cellular iron-exporting ferroxidase ceruloplasmin (Cp) have brain iron accumulation causing neurodegeneration. Here, we assessed the brains of mice with combined mutation of Cp and its homolog hephaestin. Compared to single mutants, brain iron accumulation was accelerated in double mutants in the cerebellum, substantia nigra, and hippocampus. Iron accumulated within glia, while neurons were iron deficient. There was loss of both neurons and glia. Mice developed ataxia and tremor, and most died by 9 months. Treatment with the oral iron chelator deferiprone diminished brain iron levels, protected against neuron loss, and extended lifespan. Ferroxidases play important, partially overlapping roles in brain iron homeostasis by facilitating iron export from glia, making iron available to neurons. Above: Iron (Fe) normally moves from capillaries to glia to neurons. It is exported from the glia by ferroportin (Fpn) with ferroxidases ceruloplasmin (Cp) and/or Hephaestin (Heph). Below: In mice with mutation of Cp and Heph, iron accumulates in glia, while neurons have low iron levels. Both neurons and glia degenerate and mice become ataxic unless given an iron chelator.
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Registered trials
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