AB15080 | Anti-Frataxin Antibody

AB15080
100 µL  
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      Overview

      Replacement Information

      Key Spec Table

      Species ReactivityKey ApplicationsHostFormatAntibody Type
      H, M, RWBRbPurifiedPolyclonal Antibody
      Description
      Catalogue NumberAB15080
      DescriptionAnti-Frataxin Antibody
      Background InformationFrataxin is a nuclear encoded protein that is found in the mitochondria. This protein is not well understood but may be involved with iron sulfur clusters. In humans the gene is localized on chromosome 9 and is highly conserved during evolution. The gene is expressed in every cell, although in varying levels in different tissues and during development. The specific function of Frataxin is still unknown, but it has been shown to play a role in iron metabolism. Studies have demonstrated that the deletion of the Frataxin gene in yeast results in iron accumulation in mitochondria and loss of respiration. Recombinant human Frataxin has been shown to bind iron in vitro, and increased mitochondrial iron levels have been observed in patients with Friedreich's Ataxia (FRDA).
      References
      Product Information
      FormatPurified
      Control
      • Human heart lysate.
      PresentationRabbit polyclonal serum in buffer containing 0.05% NaN3.
      Applications
      ApplicationThis Anti-Frataxin Antibody is validated for use in WB for the detection of Frataxin.
      Key Applications
      • Western Blotting
      Biological Information
      ImmunogenGST-tagged Frataxin corresponding to mature chain.
      Epitopemature chain
      HostRabbit
      SpecificityCat. # AB15080 recognizes Frataxin.
      Species Reactivity
      • Human
      • Mouse
      • Rat
      Antibody TypePolyclonal Antibody
      Entrez Gene Number
      Entrez Gene SummaryThis 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 occurs at this locus and two transcript variants encoding distinct isoforms have been identified. [provided by RefSeq]
      Purification MethodGST-depleted sera
      UniProt Number
      UniProt SummaryFunction: Probably involved in iron homeostasis. Anti-apoptotic protein which prevents mitochondrial damage and reactive oxygen species (ROS) production. Ref.13
      Subunit structure: Monomer.
      Subcellular location: Mitochondrion. Note= Mitochondrial and extramitochondrial. Ref.13 Ref.8 Ref.9
      Tissue specificity: Frataxin(81-210) is expressed in the heart, peripheral blood lymphocytes and dermal fibroblasts. Ref.4
      Post-translational modification: Processed in two steps by mitochondrial processing peptidase (MPP). MPP first cleaves the precursor to intermediate form and subsequently converts the intermediate to mature size protein. Two forms exist, frataxin(56-210) and frataxin(81-210) which is the main form of mature frataxin.
      Involvement in disease: Defects in FXN are the cause of Friedreich ataxia (FA) [MIM:229300]. FA is an autosomal recessive, progressive degenerative disease characterized by neurodegeneration and cardiomyopathy it is the most common inherited ataxia. The disorder is usually manifest before adolescence and is generally characterized by incoordination of limb movements, dysarthria, nystagmus, diminished or absent tendon reflexes, Babinski sign, impairment of position and vibratory senses, scoliosis, pes cavus, and hammer toe. In most patients, FA is due to GAA triplet repeat expansions in the first intron of the frataxin gene. But in some cases the disease is due to mutations in the coding region. Ref.6 Ref.7 Ref.16 Ref.17 Ref.18 Ref.19 Ref.20 Ref.21
      Sequence similarities: Belongs to the frataxin family.

      Molecular WeightApprox. 18 kDa
      Physicochemical Information
      Dimensions
      Materials Information
      Toxicological Information
      Safety Information according to GHS
      Safety Information
      Product Usage Statements
      Quality AssuranceRoutinely evaluated by Western Blot on Human heart lysates.

      Western Blot Analysis: 1:1,000 - 1:10,000 dilution of this lot detected Frataxin on 10 ug of human heart lysate
      Usage Statement
      • Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.
      Storage and Shipping Information
      Storage ConditionsStable for 1 year at -20ºC from date of receipt.
      Packaging Information
      Material Size100 µL
      Transport Information
      Supplemental Information
      Specifications