Decreased expression of genes involved in sulfur amino acid metabolism in frataxin-deficient cells

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Decreased expression of genes involved in sulfur amino acid metabolism in frataxin-deficient cells.

Inherited deficiency of the mitochondrial protein frataxin causes neural and cardiac cell degeneration, and Friedreich's ataxia. Five hypotheses for frataxin's mitochondrial function have been generated, largely from work in non-human cells: iron transporter, iron-sulfur cluster assembler, iron-storage protein, antioxidant and stimulator of oxidative phosphorylation. We analyzed gene expression...

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Experimental injury and sulfur amino acid metabolism.

It has been reported that methionine and cystine are capable of accelerating the healing rate of wounds in injured rats to the same extent when present in the diet in equivalent amounts (1). This response was elicited in both the presence and the absence of dietary protein (2). Lysine, tryptophan, and valine do not appear to alter the healing process to a significant degree (3). Because methion...

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Changes in Free Amino Acid Concentration in Rye Grain in Response to Nitrogen and Sulfur Availability, and Expression Analysis of Genes Involved in Asparagine Metabolism

Free asparagine plays a central role in nitrogen storage and transport in many plant species due to its relatively high ratio of nitrogen to carbon. However, it is also a precursor for acrylamide, a Class 2a carcinogen that forms during high-temperature processing and cooking. The concentration of free asparagine was shown to increase by approximately 70% in rye grain in response to severe sulf...

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RED CELLS Erythroid differentiation and protoporphyrin IX down-regulate frataxin expression in Friend cells: characterization of frataxin expression compared to molecules involved in iron metabolism and hemoglobinization

Friedreich ataxia (FA) is caused by decreased frataxin expression that results in mitochondrial iron (Fe) overload. However, the role of frataxin in mammalian Fe metabolism remains unclear. In this investigation we examined the function of frataxin in Fe metabolism by implementing a well-characterized model of erythroid differentiation, namely, Friend cells induced using dimethyl sulfoxide (DMS...

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ژورنال

عنوان ژورنال: Human Molecular Genetics

سال: 2003

ISSN: 1460-2083

DOI: 10.1093/hmg/ddg187