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抗Human Frataxin 抗体:
抗Rat (Rattus) Frataxin 抗体:
抗Mouse (Murine) Frataxin 抗体:
Human Polyclonal Frataxin Primary Antibody for WB - ABIN1881356
Tsai, Barondeau: Human frataxin is an allosteric switch that activates the Fe-S cluster biosynthetic complex. in Biochemistry 2010
Show all 4 Pubmed References
Physiological, biochemical and biophysical studies show some differences in the expression pattern, protection against oxidants and in the aggregation state of both isoforms, suggesting that the two frataxin homologs would play similar but not identical roles in plant cell metabolism.
The authors show that loss of frataxin homolog (fh) in Drosophila leads to iron toxicity, which in turn induces sphingolipid synthesis and ectopically activates 3-phosphoinositide dependent protein kinase-1 (Pdk1 (显示 PDPK1 抗体)) and myocyte enhancer factor-2 (Mef2 (显示 MYEF2 抗体)).
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.
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 (显示 LEP 抗体)) of 0.096 min (-1 (显示 CD59 抗体))
the effect of alterations in loop-1, a stretch presumably essential for FXN function, on the conformational stability and dynamics of the native state.
differentiation of these induced pluripotent stem cells into neurons also results in resilencing of the FXN gene.
The NFS1 (显示 NFS1 抗体)/ISD11 (显示 LYRM4 抗体) complex further interacts with scaffold protein (显示 HOMER1 抗体) ISCU (显示 ISCU 抗体) and regulator protein frataxin, thereby forming a quaternary complex for Fe-S cluster formation.
Molecular dynamics flexible fitting of protein structures docked into the EM map of the model revealed a [FXN(42-210)]24.[NFS1 (显示 NFS1 抗体)]24.[ISD11 (显示 LYRM4 抗体)]24.[ISCU (显示 ISCU 抗体)]24 complex, consistent with the measured 1:1:1:1 stoichiometry of its four components.
By interphase, FISH we found that in comparison to the normal Frataxin sequence the replication of expanded alleles is slowed or delayed. According to molecular combing, origins never fired within the normal Frataxin allele.
The differentially expressed FXN regulates the development of congenital heart disease (CHD (显示 CHDH 抗体)) and the differential expression was under the control of miRNA-145. These results might provide new insight into the understanding of CHD (显示 CHDH 抗体) 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 (显示 FTL 抗体) but not correlated with transferrin (显示 Tf 抗体) 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.
This study demonstrated that mouse model of Friedreich's ataxia showed that decreased grip strength endurance time , threshold of peripheral sensitivity using Von Frey monofilaments and gait parameters.
Here, the authors show that loss of Fxn in the nervous system in mice also activates an iron/sphingolipid/PDK1 (显示 PDPK1 抗体)/Mef2 (显示 MEF2C 抗体) pathway, indicating that the mechanism is evolutionarily conserved.
The in vitro antioxidant treatments trigger the axonal re-growth and the increase in stable MTs (显示 NEU2 抗体) in shFxn, thus contributing to identify new neuronal targets of oxidation in this disease and providing a novel approach for antioxidant therapies.
Retinal FXN levels are increased in response to ischemia. Furthermore, elevated FXN levels had a clear neuroprotective effect as shown by increased ganglion cell survival after acute retinal ischemia/reperfusion. Frataxin's neuroprotective effect was associated with an upregulation of antioxidative enzymes.
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 (显示 ACO1 抗体), a key regulator of cellular iron metabolism, we show that IRP1 (显示 ACO1 抗体) activation in conditions of Fe-S deficiency increases the available cytosolic labile iron pool
The Fxn KO/Mck (显示 CKM 抗体) 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 leads to elevation of COX2 (显示 COX2 抗体)-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.
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