Frataxin overexpressing mice
Abstract
Friedreich ataxia, the most common autosomal recessive ataxia, is caused by frataxin deficiency. Reduction of frataxin has been associated with iron accumulation and sensitivity to iron induced oxidative stress. To better understand the function of frataxin, transgenic mice (tgFxn) overexpressing human frataxin were generated. Iron metabolism parameters in tgFxn were normal and no signs of ataxia or other obvious abnormalities were observed, indicating that overexpression of frataxin in mouse is innocuous. Several hypotheses for frataxin function were evaluated in tgFxn mice. In particular, we observed that TgFxn mice show an altered response during hematopoietic differentiation, suggesting that frataxin may directly affect heme synthesis.
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Oligodendrocytes have equal amounts of both H and L subunits, microglia express L-rich ferritin, and neurons have H-subunit abundant ferritin.
“Oligodendrocytes have equal amounts of both H and L subunits, whereas microglia express L-rich ferritin, and neurons have H-subunit abundant ferritin (206, 333).”
From Intracellular iron transport and storage: from molecular mechanisms to health implications (2008) · cites this paper as [206]
Many of the proteins involved in iron homeostasis throughout the body are also present in the brain.
“It is apparent that many of the proteins involved in iron homeostasis throughout the body are also present in the brain (206).”
From Intracellular iron transport and storage: from molecular mechanisms to health implications (2008) · cites this paper as [206]
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- Authors
- Miranda, C. J., Santos, M. M., Ohshima, K., Tessaro, M., Sequeiros, J., & Pandolfo, M.
- Affiliations
- Universidade do Porto (PT)
- Hôpital Notre-Dame (CA)
- Université Libre de Bruxelles (BE)
- Corresponding author
- Pandolfo, Massimo
- Venue
- FEBS Letters
- Publisher
- Wiley
- Published
- 2004-07-22
- Volume
- 572
- Issue
- 1-3
- Pages
- 281-288
- ISSN
- 0014-5793 (ISSN-L)
- 1873-3468
- OpenAlex
- W1966828804
- Cited by
- 30
- References
- 50
- Citations by year
- 2022: 1 · 2020: 1 · 2019: 1 · 2018: 3 · 2017: 3 · 2016: 2 · 2015: 1 · 2014: 2
- FWCI
- 1.29
- Type
- journal article
- Language
- en
- Open access
- closed
- Locations
- FEBS Letters (publishedVersion)
- PubMed (publishedVersion)
- Dépôt institutionnel de l'Université libre de Bruxelles (Université Libre de Bruxelles) (submittedVersion)
- Funders
- National Institutes of Health (7r01ns034192-06)
- National Institute of Neurological Disorders and Stroke (R01-NS34192)
- Topics
- Life Sciences › Neuroscience › Cellular and Molecular Neuroscience › Genetic Neurodegenerative Diseases
- Health Sciences › Medicine › Hematology › Iron Metabolism and Disorders
- Life Sciences › Neuroscience › Neurology › Neurological diseases and metabolism
- Keywords
- frataxin, Friedreich's ataxia, transgenic mice, iron accumulation, oxidative stress, iron metabolism, heme biosynthesis, hematopoietic differentiation, autosomal recessive ataxia
- MeSH terms
- Frataxin, Animals, Erythrocyte Count, Friedreich Ataxia, Hematocrit, Hematopoiesis, Humans, Iron, Mice, Mutant Strains, Mice, Transgenic, Transferrin, Polymerase Chain Reaction, DNA Primers, Iron-Binding Proteins, Mice
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