Title
Role of Frataxin in Mitochondrial Iron and Haem Metabolism: Development of Potential Iron Chelators for the Treatment of Friedre,Used
Delivery time: 8-12 business days (International)
Friedreich's ataxia (FA) is a severe neurodegenerative condition with an incidence of 1:50,000 in the European population. The defective protein encoded by the FRDA gene is known as frataxin. While the precise role of human frataxin remains to be determined, it appears to be involved in regulating mitochondrial Fe. We examined frataxin in Fe metabolism by implementing a model of erythroid differentiation. Since decreased frataxin expression leads to mitochondrial Fe loading in FA, our data suggest that reduced frataxin expression results in mitochondrial Fe sequestration for heme biosynthesis. These findings suggest that the use of specific Fe chelators may have potential in the treatment of this disease. In order to develop effective (Fe) chelators for FA, we synthesized and characterised three classes of aroylhydrazone analogues based on: (1) 2pyridylcarboxaldehyde isonicotinoyl hydrazone (PCIH), (2) 2quinoline carboxaldehyde isonicotinoyl hydrazone (QCIH), and (3) di2 pyridylketone isonicotinoyl hydrazone (PKIH). This study identified not only structural features useful in the logical design of chelators for the treatment of FA.
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Shipping & Returns
Shipping
We ship your order within 2–3 business days for USA deliveries and 5–8 business days for international shipments. Once your package has been dispatched from our warehouse, you'll receive an email confirmation with a tracking number, allowing you to track the status of your delivery.
Returns
To facilitate a smooth return process, a Return Authorization (RA) Number is required for all returns. Returns without a valid RA number will be declined and may incur additional fees. You can request an RA number within 15 days of the original delivery date. For more details, please refer to our Return & Refund Policy page.
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Warranty
We provide a 2-year limited warranty, from the date of purchase for all our products.
If you believe you have received a defective product, or are experiencing any problems with your product, please contact us.
This warranty strictly does not cover damages that arose from negligence, misuse, wear and tear, or not in accordance with product instructions (dropping the product, etc.).
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Your payment information is processed securely. We do not store credit card details nor have access to your credit card information.
We accept payments with :
Visa, MasterCard, American Express, Paypal, Shopify Payments, Shop Pay and more.