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In vivo analysis of the mitochondrial protein frataxin in Drosophila melanogaster

URN to cite this document:
urn:nbn:de:bvb:355-epub-364791
DOI to cite this document:
10.5283/epub.36479
Edenharter, Oliver Manfred
Date of publication of this fulltext: 02 Jul 2018 08:25


Abstract (English)

Friedreich’s ataxia is the most common recessive ataxia in the Caucasian population. It is caused by a defect in the expression of the mitochondrial protein frataxin and leads to neurodegeneration. The main role of frataxin lies in iron-sulphur cluster biosynthesis and loss of the protein results in mitochondrial iron accumulation and diminished energy production within the cell. Although ...

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Translation of the abstract (German)

Die Friedreich-Ataxie ist die häufigste rezessive Ataxie bei Weißen. Sie wird durch einen Defekt in der Expression des mitochondriellen Proteins Frataxin hervorgerufen und führt zu Neurodegeneration. Die Hauptrolle von Frataxin liegt in der Biogenese von Eisen-Schwefel-Clustern, wobei der Verlust des Proteins zu Eisenakkumulation in Mitochondrien und verringerter Energieproduktion führt. Obwohl ...

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