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Browse our Frataxin Proteins (FXN)

Full name:
Frataxin Proteins (FXN)
On www.antibodies-online.com are 31 Frataxin (FXN) Proteins from 8 different suppliers available. Additionally we are shipping Frataxin Antibodies (139) and Frataxin Kits (9) and many more products for this protein. A total of 188 Frataxin products are currently listed.
Synonyms:
BcDNA:AT09528, CG8971, CyaY, dfh, Dmel\\CG8971, FA, FARR, Frda, LOC100284365, RGD1565754, X25, zgc:158218
list all proteins Gene Name GeneID UniProt
FXN 2395 Q16595
FXN 499335  
FXN 14297 O35943

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Frataxin Proteins (FXN) by Origin

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Top referenced Frataxin Proteins

  1. Human Frataxin Protein expressed in Escherichia coli (E. coli) - ABIN667758 : Campuzano, Montermini, Moltò, Pianese, Cossée, Cavalcanti, Monros, Rodius, Duclos, Monticelli, Zara, Cañizares, Koutnikova, Bidichandani, Gellera, Brice, Trouillas, De Michele, Filla, De Frutos et al.: Friedreich's ataxia: autosomal recessive disease caused by an intronic GAA triplet repeat expansion. ... in Science (New York, N.Y.) 1996 (PubMed)
    Show all 2 references for ABIN667758

More Proteins for Frataxin Interaction Partners

Fruit Fly (Drosophila melanogaster) Frataxin (FXN) interaction partners

  1. Frataxin overexpression in the nervous system reduces life span, impairs locomotor ability and causes brain degeneration.

  2. Strong involvement of glial cells and lipid peroxidation in the generation of Friedreich's ataxia (show USP14 Proteins).

  3. Defects in axonal transport of mitochondria appear late in development in distal nerve of larvae showing reduced frataxin expression, with retrograde movement preferentially affected.

  4. H2O2 is an important pathogenic substrate underlying the phenotypes arising from frataxin deficiency in Drosophila

  5. This suggests that Drosophila frataxin may function to protect the mitochondria from oxidative stresses and the ensuing cellular damage.

  6. Dfh-assisted assembly of Fe-S clusters occurs with an observed kinetic rate constant ( k obs (show LEP Proteins)) of 0.096 min (-1 (show CD59 Proteins))

Human Frataxin (FXN) interaction partners

  1. The NFS1 (show NFS1 Proteins)/ISD11 (show LYRM4 Proteins) complex further interacts with scaffold protein (show HOMER1 Proteins) ISCU (show ISCU Proteins) and regulator protein frataxin, thereby forming a quaternary complex for Fe-S cluster formation.

  2. Molecular dynamics flexible fitting of protein structures docked into the EM map of the model revealed a [FXN(42-210)]24.[NFS1 (show NFS1 Proteins)]24.[ISD11 (show LYRM4 Proteins)]24.[ISCU (show ISCU Proteins)]24 complex, consistent with the measured 1:1:1:1 stoichiometry of its four components.

  3. 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.

  4. The differentially expressed FXN regulates the development of congenital heart disease (CHD (show CHDH Proteins)) and the differential expression was under the control of miRNA-145. These results might provide new insight into the understanding of CHD (show CHDH Proteins) pathogenesis and may facilitate further therapeutic studies.

  5. 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.

  6. relative FXN expression in the patients was found to be correlated with the levels of MDA and ferritin (show FTL Proteins) but not correlated with transferrin (show Tf Proteins) saturation

  7. Frataxin (FXN) gene mutations lead to mitochondrial iron accumulation without total body/organ iron overload. The clinical consequences are spinocerebellar degeneration and frequent cardiomyopathy.

  8. 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.

  9. 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.

  10. 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.

Mouse (Murine) Frataxin (FXN) interaction partners

  1. The in vitro antioxidant treatments trigger the axonal re-growth and the increase in stable MTs (show NEU2 Proteins) in shFxn, thus contributing to identify new neuronal targets of oxidation in this disease and providing a novel approach for antioxidant therapies.

  2. 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.

  3. Frataxin Deficiency Promotes Excess Microglial DNA Damage and Inflammation that Is Rescued by PJ34

  4. Frataxin-deficient mice, which had higher mitochondrial iron loading, showed impaired airway mucociliary clearance and higher pulmonary inflammation at baseline.

  5. Using a mouse model of hepatic FXN deficiency in combination with mice deficient for IRP1 (show ACO1 Proteins), a key regulator of cellular iron metabolism, we show that IRP1 (show ACO1 Proteins) activation in conditions of Fe-S deficiency increases the available cytosolic labile iron pool

  6. The Fxn KO/Mck (show CKM Proteins) mice tested from one to two months of age showed abnormal gait patterns accompanied by a loss in motor skills

  7. Reduced expression of frataxin in Friedreich's ataxia (show USP14 Proteins) leads to elevation of COX2-mediated oxylipin synthesis stimulated by increases in transcription factors that respond to increased reactive oxygen species.

  8. 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.

  9. 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.

  10. rescue of the Friedreich ataxia knockout mutation in transgenic mice containing an FXN-EGFP genomic reporter

Frataxin (FXN) Protein Profile

Protein Summary

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.

Alternative names and synonyms associated with Frataxin (FXN)

  • frataxin (fxn)
  • frataxin (LOC100284365)
  • frataxin (TVAG_182150)
  • frataxin homolog (fh)
  • frataxin (FTX1)
  • frataxin (FXN)
  • frataxin (Fxn)
  • BcDNA:AT09528 protein
  • CG8971 protein
  • CyaY protein
  • dfh protein
  • Dmel\\CG8971 protein
  • FA protein
  • FARR protein
  • Frda protein
  • LOC100284365 protein
  • RGD1565754 protein
  • X25 protein
  • zgc:158218 protein

Protein level used designations for Frataxin Proteins (FXN)

DKEYP-118A9.1 , frataxin, mitochondrial , frataxin , CG8971-PA , CG8971-PB , fh-PA , fh-PB , frataxin homologue , frataxin-like , Friedreich ataxia protein , Friedreich ataxia

GENE ID SPECIES
556389 Danio rerio
100284365 Zea mays
5468558 Trichomonas vaginalis G3
31845 Drosophila melanogaster
5718448 Chlamydomonas reinhardtii
2395 Homo sapiens
499335 Rattus norvegicus
14297 Mus musculus
505694 Bos taurus
427244 Gallus gallus
609051 Canis lupus familiaris
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