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Showing 1 to 11 of 11 for “"frataxin"”.
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Identifikation des mitochondrialen Proteins Frataxin als stoffwechselmodulierenden Tumorsuppressor
… Expression des mitochondrialen Proteins Frataxin konnte in der vorliegenden Arbeit gezeigt werden, dass der mitochondriale Energiestoffwechsel einen Einfluss auf die Tumorentstehung zu besitzen scheint. Eine Reduktion des mitochondrialen Energiestoffwechsels wurde durch die …
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Definition of The Landscape of Chromatin Structure At The Frataxin Gene In Friedreich’S Ataxia
… caused by the transcriptional silencing of the frataxin (<em>FXN</em>) gene. FRDA patients have expansion of GAA repeats in intron 1 of the <em>FXN</em> gene in both alleles. A number of studies demonstrated that specific histone deacetylase inhibitors (HDACi) affect either histone modifications …
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Molecular Details Of The Mitochondrial Iron Sulfur Cluster Assembly Pathway
… by deficiency of a single protein called as `Frataxin'. Frataxin is a mitochondrial protein shown to regulate cellular iron homeostasis. Frataxin is highly conserved from prokaryotes to eukaryotes but its cellular function has not been completely characterized.</p> <p>The focus of the research …
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Characterization Of Initial Iron Binding Location And The Structure/iron Binding Site On S.cerevisiae Isu And On D.melanogaster Frataxin
… to synthesize the mitochondrial protein, frataxin. Frataxin has been shown by numerous groups to be a part of the iron-sulfur cluster (ISC) multicomplex, where it functions in the capacity of a potential iron provider and an allosteric modulator of both the cysteine desulfurase and …
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Isothermal Titration Calorimetry Studies Of Protein-Mediated Interactions And Preliminary Structural Studies Of Tandem Pdz1-2 Domain Of Psd-95 Protein
… for two proteins, the PDZ domain and frataxin, involving peptide and metal binding ligands, respectively. The three component projects of this work comprised (1) thermodynamic analysis of PDZ domain binding using calorimetry; (2) X-ray crystallographic structural studies of a PDZ dual …
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Unraveling dual dysfunction in Friedreich’s ataxia: how mitochondrial dysfunction and microglial activation drive neurodegeneration
… FXN gene which leads to reduced expression of frataxin. It has been speculated that mitochondrial function could be contributing to FRDA. Frataxin is a mitochondrial protein crucial for iron-sulfur cluster biogenesis and mitochondrial function. When frataxin levels are depleted, mitochondrial …
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Therapeutic testing and epigenetic characterization of Friedreich Ataxia
… a deficiency of the mitochondrial protein frataxin. In the first instance, frataxin deficiency renders an impaired protection from oxidative stress. Antioxidant therapy with cannabinoids (CBD and THC) and CTMIO was investigated in GAA repeat FXN YAC transgenic mouse models of FRDA, but no …
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A Protective Role Of Autophagy In A Drosophila Model Of Friedreich's Ataxia (frda)
… North America. FRDA is caused by deficiency of Frataxin, an essential mitochondrial iron chaperone protein, and the associated oxidative stress damages. Autophagy, a housekeeping process responsible for the bulk degradation and turnover of long half-life proteins and organelles, is featured by …
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EXPLORING MITOCHONDRIAL AND REDOX VULNERABILITY IN FRIEDREICH'S ATAXIA USING GOLD NANOCLUSTERS IN HUMAN CELLULAR MODELS
… silencing of the FXN gene, leading to frataxin deficiency, impaired iron–sulfur cluster biogenesis, mitochondrial dysfunction, and chronic oxidative stress. Despite significant advances in the understanding of FRDA pathophysiology, the identification of reliable cellular models for …
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Mitochondrial protein acetylation and left ventricular function in a model of hypertrophic cardiomyopathy and heart failure
… hypertrophy and failure. It is caused by loss of frataxin (FXN), a mitochondrial protein involved in energy homeostasis. FRDA model hearts have increased mitochondrial protein acetylation and impaired sirtuin 3 (SIRT3) deacetylase activity. Protein acetylation is an important regulator of cardiac …
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Investigating the pathogenesis and therapy of Friedreich Ataxia
… expression. Nevertheless, elevated levels of frataxin protein in the brain tissues were obtained with RGFP 109, suggesting that RGFP 109 is capable of crossing the blood-brain barrier. I have also found increased levels of global acetylated H3 and H4 histone proteins in brain tissues, along …