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.
Publication types
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Comparative Study
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Research Support, Non-U.S. Gov't
MeSH terms
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Animals
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Blotting, Western
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Dose-Response Relationship, Drug
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Enzyme Inhibitors / pharmacology*
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Frataxin
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Friedreich Ataxia / metabolism
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Humans
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Iron-Binding Proteins / biosynthesis*
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Iron-Binding Proteins / drug effects*
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Iron-Binding Proteins / genetics
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Lymphocytes / drug effects*
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Nitro Compounds
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Oxidative Stress
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PC12 Cells
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Propionates / pharmacology*
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Rats
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Stem Cells / drug effects
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Time Factors
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Transgenes
Substances
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Enzyme Inhibitors
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Iron-Binding Proteins
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Nitro Compounds
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Propionates
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3-nitropropionic acid