Definitions
from Wiktionary, Creative Commons Attribution/Share-Alike License.
- noun biochemistry A class of
protein found inmitochondria
Etymologies
Sorry, no etymologies found.
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Examples
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In any case, when frataxin is missing or defective, free iron accumulates in mitochondria and oxidative stress damages the mitochondria.
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FA patients have gene mutations that limit the production of a protein call frataxin, an important protein that functions in the (Technical Difficulty) - and thus affects energy function.
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One theory holds that frataxin acts like a storage depot for iron, releasing it only when needed.
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Scientists believe the frataxin protein regulates the levels of iron inside mitochondria — tiny cellular powerhouses that use oxygen to produce energy.
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FA is caused by defects, or mutations, in the gene coding frataxin.
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To see if targeting these enzymes would result in an increase in frataxin, the researchers tested various commercially available HDAC inhibitors and found only one that was slightly active on the frataxin gene (compound BML-210).
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The interaction between the active form of the frataxin gene and the inactive (silenced) form is mediated by enzymes called histone deacetylases (HDAC).
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To see if targeting these enzymes would result in an increase in frataxin, the researchers tested various commercially available HDAC inhibitors and found only one that was slightly active on the frataxin gene (compound BML-210).
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Friedreich's ataxia is a congenital neurodegenerative disease caused by a gene defect that leads to a deficiency in the protein frataxin, leading to progressive damage to the nervous system and loss of muscle function.
Boston Business News - Local Boston News | Boston Business Journal 2010
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Preclinical studies have shown that specific HDAC-3 inhibitors increase production of the protein frataxin which may have the potential to arrest disease progression in patients with Friedreich's ataxia.
TradingMarkets 2010
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