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AB15080 Anti-Frataxin Antibody

This Anti-Frataxin Antibody is validated for use in WB for the detection of Frataxin.

Anti-Frataxin Antibody MSDS (material safety data sheet) or SDS, CoA and CoQ, dossiers, brochures and other available documents.
Research Category: Neuroscience Research Sub-Category: Neurodegenerative Diseases UniProt Number: Q16595
AB15080
100 µL  
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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.
Quality LevelMQ100
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
Global Trade Item Number
Catalogue Number GTIN
AB15080 04053252277689