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This nuclear gene encodes a mitochondrial protein which belongs to FRATAXIN family. Additionally we are shipping Frataxin Antibodies (139) and Frataxin Proteins (35) and many more products for this protein.
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Frataxin overexpression in the nervous system reduces life span, impairs locomotor ability and causes brain degeneration.
Strong involvement of glial cells and lipid peroxidation in the generation of Friedreich's ataxia (show USP14 ELISA Kits).
Defects in axonal transport of mitochondria appear late in development in distal nerve of larvae showing reduced frataxin expression, with retrograde movement preferentially affected.
H2O2 is an important pathogenic substrate underlying the phenotypes arising from frataxin deficiency in Drosophila
This suggests that Drosophila frataxin may function to protect the mitochondria from oxidative stresses and the ensuing cellular damage.
Dfh-assisted assembly of Fe-S clusters occurs with an observed kinetic rate constant ( k obs (show LEP ELISA Kits)) of 0.096 min (-1 (show CD59 ELISA Kits))
The differentially expressed FXN regulates the development of congenital heart disease (CHD (show CHDH ELISA Kits)) and the differential expression was under the control of miRNA-145. These results might provide new insight into the understanding of CHD (show CHDH ELISA Kits) pathogenesis and may facilitate further therapeutic studies.
Results presented here shed light on the folding mechanism of frataxin, opening the possibility of mutating it to generate hyperstable variants without altering their folding kinetics.
relative FXN expression in the patients was found to be correlated with the levels of MDA and ferritin (show FTL ELISA Kits) but not correlated with transferrin (show Tf ELISA Kits) saturation
Frataxin (FXN) gene mutations lead to mitochondrial iron accumulation without total body/organ iron overload. The clinical consequences are spinocerebellar degeneration and frequent cardiomyopathy.
Our results imply that regulation of FXN protein levels is complex and that total amounts can be modulated chemically and genetically without altering the absolute amount of mature FXN protein.
In compound heterozygotes, expression of partially functional mutant frataxin delays age of onset and reduces diabetes mellitus, compared to those with no frataxin expression from the non-expanded allele.
FXN promoter function was directly measured via metabolic labeling of newly synthesized transcripts in fibroblasts, which revealed that the YG8sR mouse was significantly deficient in transcriptional initiation compared to the Y47R mouse.
Thus, Src (show SRC ELISA Kits) inhibitors emerge as a new class of drugs able to promote frataxin accumulation, suggesting their possible use as therapeutics in Friedreich's Ataxia (show USP14 ELISA Kits)
Engineered a cell line where the presence of an exogenous, inducible FXN gene rescues the cells from the knockout of the two endogenous FXN genes. This system allows the possibility of testing the progression of disease.
The region of chromosome 9 carrying the FXN gene is prone to chromosomal rearrangements in both control and Friedreich ataxia patient cells.
Frataxin Deficiency Promotes Excess Microglial DNA Damage and Inflammation that Is Rescued by PJ34
Frataxin-deficient mice, which had higher mitochondrial iron loading, showed impaired airway mucociliary clearance and higher pulmonary inflammation at baseline.
Using a mouse model of hepatic FXN deficiency in combination with mice deficient for IRP1 (show ACO1 ELISA Kits), a key regulator of cellular iron metabolism, we show that IRP1 (show ACO1 ELISA Kits) activation in conditions of Fe-S deficiency increases the available cytosolic labile iron pool
The Fxn KO/Mck (show CKM ELISA Kits) mice tested from one to two months of age showed abnormal gait patterns accompanied by a loss in motor skills
Reduced expression of frataxin in Friedreich's ataxia (show USP14 ELISA Kits) leads to elevation of COX2 (show COX2 ELISA Kits)-mediated oxylipin synthesis stimulated by increases in transcription factors that respond to increased reactive oxygen species.
Frataxin-deficient cells showed a specific inhibition of mitochondrial Complex I activity already at 70% residual frataxin levels, whereas the glutathione imbalance progressively increased after silencing.
The results support a mechanistic hypothesis in which frataxin deficiency decreases Nrf2 expression in vivo, causing the sensitivity to oxidative stress in target tissues the DRG and the cerebella, which contributes to the process of neurodegeneration.
rescue of the Friedreich ataxia knockout mutation in transgenic mice containing an FXN-EGFP genomic reporter
these results indicate that IGF-I (show IGF1 ELISA Kits) exerts cell-context neuroprotection in frataxin deficiency that maybe therapeutically effective.
Data show that the respiratory chain defects accompanying frataxin deficiency cause progressive hyperacetylation of cardiac mitochondrial proteins due to the inhibition of SIRT3 (show SIRT3 ELISA Kits) deacetylase.
This nuclear gene encodes a mitochondrial protein which belongs to FRATAXIN family. The protein functions in regulating mitochondrial iron transport and respiration. The expansion of intronic trinucleotide repeat GAA results in Friedreich ataxia. Alternative splicing results in multiple transcript variants.
, frataxin, mitochondrial
, frataxin homologue
, Friedreich ataxia protein
, Friedreich ataxia