3-Nitropropionic acid increases frataxin expression in human lymphoblasts and in transgenic rat PC12 cells
Abstract
Friedreich ataxia (FRDA) is the most common recessive ataxia caused by reduced expression of frataxin, a nuclear encoded mitochondrial protein. In this study we examined the effects of 3-nitropropionic acid (3-NP) on frataxin expression in FRDA patient and control lymphoblasts and in rat pheochromocytoma cell line (PC12) overexpressing human frataxin. Our studies showed an up-regulation of frataxin expression in both FRDA and control lymphoblasts following exposure to 3-NP. In addition, in transgenic frataxin overexpressing cells 3-NP caused an increase of frataxin protein.
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Heph was identified through study of sex-linked anemia (Sla) mice, which are deficient in transfer of iron absorbed in the intestines into the plasma.
“Heph was identified through study of sex-linked anemia (Sla) mice, which are deficient in transfer of iron absorbed in the intestines into the plasma (313).”
From Intracellular iron transport and storage: from molecular mechanisms to health implications (2008) · cites this paper as [313]
In the intestine, where Cp is not expressed, Fpn couples with another multicopper oxidase, hephaestin (Heph).
“In the intestine, where Cp is not expressed, Fpn couples with another multicopper oxidase, hephaestin (Heph) (313).”
From Intracellular iron transport and storage: from molecular mechanisms to health implications (2008) · cites this paper as [313]
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Intracellular iron transport and storage: from molecular mechanisms to health implications
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- Authors
- Turano, M., Tammaro, A., De Biase, I., Lo Casale, M. S., Ruggiero, G., Monticelli, A., Cocozza, S., & Pianese, L.
- Affiliations
- Institute for Experimental Endocrinology and Oncology (IT)
- Ospedale Antonio Cardarelli (IT)
- Corresponding author
- Turano, Mimmo
- Venue
- Neuroscience Letters
- Publisher
- Elsevier BV
- Published
- 2003-10
- Volume
- 350
- Issue
- 3
- Pages
- 184-186
- ISSN
- 0304-3940 (ISSN-L)
- OpenAlex
- W2080077726
- Cited by
- 17
- References
- 20
- Citations by year
- 2019: 1 · 2017: 1
- FWCI
- 0.58
- Type
- journal article
- Language
- en
- Open access
- closed
- Locations
- Neuroscience Letters (publishedVersion)
- PubMed (publishedVersion)
- Topics
- Life Sciences › Neuroscience › Cellular and Molecular Neuroscience › Genetic Neurodegenerative Diseases
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Mitochondrial Function and Pathology
- Life Sciences › Neuroscience › Cellular and Molecular Neuroscience › Hereditary Neurological Disorders
- Keywords
- 3-nitropropionic acid, frataxin, Friedreich's ataxia, lymphoblasts, PC12 cells, transgenic rats, mitochondrial proteins, neurotoxicity
- MeSH terms
- Frataxin, Animals, Dose-Response Relationship, Drug, Enzyme Inhibitors, Friedreich Ataxia, Humans, Lymphocytes, Nitro Compounds, Propionates, Stem Cells, Time Factors, Blotting, Western, PC12 Cells, Oxidative Stress, Transgenes, Iron-Binding Proteins, Rats
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