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Iron metabolism in mice with partial frataxin deficiency
The Cerebellum, 2003Friedreich ataxia (FRDA), the most common autosomal recessive inherited ataxic disorder, is the consequence of deficiency of the mitochondrial protein frataxin, typically caused by homozygous intronic GAA expansions in the corresponding gene. The yeast frataxin homologue (yfh1p) is required for cellular respiration.
Santos, M M +8 more
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Molecular Nutrition & Food Research, 2018
SCOPE Naturally occurring quercetin has been found to induce mitophagy and prevent nonalcoholic fatty liver disease (NAFLD). However, it still remains elusive whether frataxin upregulation by quercetin contributes to the beneficial effect through ...
Peiyi Liu +9 more
semanticscholar +1 more source
SCOPE Naturally occurring quercetin has been found to induce mitophagy and prevent nonalcoholic fatty liver disease (NAFLD). However, it still remains elusive whether frataxin upregulation by quercetin contributes to the beneficial effect through ...
Peiyi Liu +9 more
semanticscholar +1 more source
Frataxin: a protein in search for a function
Journal of Neurochemistry, 2013AbstractReduced levels of the protein frataxin cause the neurodegenerative disease Friedreich's ataxia. Pathology is associated with disruption of iron–sulfur cluster biosynthesis, mitochondrial iron overload, and oxidative stress. Frataxin is a highly conserved iron‐binding protein present in most organisms.
Annalisa Pastore, Helene Puccio
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Frataxin Deacetylation in Macrophages: Avoiding SIRTain Myocyte Death
Circulation Research, 2023Ronald J. Vagnozzi, Emma L. Robinson
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