Definitions

from Wiktionary, Creative Commons Attribution/Share-Alike License

  • n. A class of protein found in mitochondria

Etymologies

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Examples

  • In any case, when frataxin is missing or defective, free iron accumulates in mitochondria and oxidative stress damages the mitochondria.

    Friedreich's Ataxia: Causes

  • 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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  • FA is caused by defects, or mutations, in the gene coding frataxin.

    Friedreich's Ataxia: Causes

  • One theory holds that frataxin acts like a storage depot for iron, releasing it only when needed.

    Friedreich's Ataxia: Causes

  • Scientists believe the frataxin protein regulates the levels of iron inside mitochondria — tiny cellular powerhouses that use oxygen to produce energy.

    Friedreich's Ataxia: Causes

  • 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.

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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.

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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).

    WN.com - Business News

  • However they were then able to study derivatives of this compound and identified a compound (4B) which is active at increasing levels of frataxin mRNA and frataxin protein in cells.

    WN.com - Business News

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