MAB1594 Anti-Frataxin Antibody, exon 4, clone 1G2

MAB1594
100 µL  
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      Overview

      Replacement Information

      Key Specifications Table

      Species ReactivityKey ApplicationsHostFormatAntibody Type
      H, M, R ELISA, ICC, IF, WB M Ascites Monoclonal Antibody
      Description
      Catalogue NumberMAB1594
      Brand Family Chemicon®
      Trade Name
      • Chemicon
      DescriptionAnti-Frataxin Antibody, exon 4, clone 1G2
      Alternate Names
      • Friedreich ataxia
      • Friedreich ataxia protein
      Background InformationFrataxin is a monomeric mitochondrial protein that is believed to be involved in iron homeostasis through an unknown mechanism. Expression of frataxin is highest in tissue rich with mitochondria including liver, heart, and skeletal muscle (Campuzano, 1996; Koutnikova, 1997). Frataxin is expressed as a 30 kDa precursor (transient; 210 amino acids) that is processed within in the mitochondria in two steps catalysed by the mitochondrial processing peptidase (MPP) to yield the mature protein (Koutnikova, 1998). The first step involves cleavage of the first 41 N-terminal amino acids by MPP yielding a transient intermediate of approximately 20 kDa (aa 42-210). Further cleavage of the N-terminus of this 20 kDa intermediate by MPP results in the mature 18 kDa frataxin protein (aa 56-210). Defects in the gene encoding frataxin are implicated as the cause of Friedreich's ataxia, an autosomal recessive, progressive degenerative disease characterized by neurodegeneration and cardiomyopathy. In the majority of cases of Friedreich's ataxia, there is an expansion of a trinucleotide repeat in the first intron of the gene encoding frataxin resulting in a marked decrease in frataxin expression, perhaps due to the formation of some unusual yet stable DNA structure that interferes with transcription (Campuzano, 1997; Bidichandani, 1998). This reduction in frataxin expression results in the accumulation of unchelated iron in the mitochondria, inhibition of mitochondrial iron-sulfer proteins, and iron mediated oxidative stress (Foury, 1997; for review see Puccio, 2000).
      References
      Product Information
      FormatAscites
      Control
      • Liver, heart or skeletal muscle.
      PresentationMouse monoclonal ascites IgG1κ in buffer containing liquid with no preservatives.
      Applications
      ApplicationDetect Frataxin using this Anti-Frataxin Antibody, exon 4, clone 1G2 validated for use in ELISA, IC, IF & WB.
      Key Applications
      • ELISA
      • Immunocytochemistry
      • Immunofluorescence
      • Western Blotting
      Application NotesImmunofluorescence:
      1:100-1:1,000. Fixation of cells in ice cold acetone or 4% paraformaldehyde is recommended. Due to the subcellular localization of frataxin in the mitochondria, cells should be permeabilized in the presence of detergent prior to incubation with primary antibody.

      ELISA:
      A previous lot of this antibody was used on ELISA.

      Western blot (natural and recombinant protein):
      1:5,000; mitochondrial preparations are recommended for consist signals (see Santos, 2001).

      Optimal working dilutions must be determined by the end user.
      Biological Information
      ImmunogenFull length human Frataxin fused to TrpE.
      Epitopeexon 4
      Clone1G2
      ConcentrationPlease refer to the Certificate of Analysis for the lot-specific concentration.
      HostMouse
      SpecificityHuman Frataxin. MAB1594 recognizes only isoforms of frataxin containing exon 4. On Western blots of normal human muscle, heart, cerebellum, and spinal cord extracts, MAB1594 recognizes a band migrating at approximately 18 kDa corresponding to processed frataxin (Campuzano, 1997). Slight cross reactivity with myosin may be observed by Western blot. Immunofluorescent labeling of HeLa cells with MAB1594 demonstrates that frataxin is predominantly localized in the mitochondria (Campuzano, 1997).
      IsotypeIgG1κ
      Species Reactivity
      • Human
      • Mouse
      • Rat
      Species Reactivity NoteMouse and rat. Expected to cross-react with human.
      Antibody TypeMonoclonal 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.
      Gene Symbol
      • FXN
      • FRDA
      • FARR
      • FA
      • Fxn
      • MGC57199
      • CyaY
      • X25
      Purification MethodUnpurified
      UniProt Number
      UniProt SummaryFUNCTION: SwissProt: Q16595 # Probably involved in iron homeostasis. Anti-apoptotic protein which prevents mitochondrial damage and reactive oxygen species (ROS) production.
      SIZE: 210 amino acids; 23135 Da
      SUBUNIT: Monomer.
      SUBCELLULAR LOCATION: Mitochondrion. Note=Mitochondrial and extramitochondrial.
      TISSUE SPECIFICITY: Frataxin(81-210) is expressed in the heart, peripheral blood lymphocytes and dermal fibroblasts.
      PTM: 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.
      DISEASE: SwissProt: Q16595 # 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.
      SIMILARITY: SwissProt: Q16595 ## Belongs to the frataxin family
      Molecular Weight~ 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 PC12 lysates.

      Western Blot Analysis:
      1:1000 dilution of this lot detected Frataxin on 10 μg of PC12 lysates.
      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 years at -20°C in undiluted aliquots from date of receipt.
      Handling Recommendations: Upon first thaw, and prior to removing the cap, centrifuge the vial and gently mix the solution. Aliquot into microcentrifuge tubes and store at -20°C. Avoid repeated freeze/thaw cycles, which may damage IgG and affect product performance
      Packaging Information
      Material Size100 µL
      Transport Information
      Supplemental Information
      Specifications

      Documentation

      SDS

      Title

      Safety Data Sheet (SDS) 

      Certificates of Analysis

      TitleLot Number
      Anti-Frataxin, exon 4, clone 1G2 - 24467112446711
      Anti-Frataxin, exon 4, clone 1G2 - 23435452343545
      Anti-Frataxin, exon 4, clone 1G2 - JC1594173JC1594173
      Anti-Frataxin, exon 4, clone 1G2 - LV1438389LV1438389
      Anti-Frataxin, exon 4, clone 1G2 - NG1713428NG1713428
      Anti-Frataxin, exon 4, clone 1G2 - NG1817595NG1817595
      Anti-Frataxin, exon 4, clone 1G2 -25063012506301
      Anti-Frataxin, exon 4, clone 1G2 -26794652679465
      Anti-Frataxin, exon 4, clone 1G2 -26891452689145
      Anti-Frataxin, exon 4, clone 1G2 -27465042746504

      References

      Reference overviewApplicationSpeciesPub Med ID
      A high throughput electrochemiluminescence assay for the quantification of frataxin protein levels.
      Hannes Steinkellner,Barbara Scheiber-Mojdehkar,Hans Goldenberg,Brigitte Sturm
      Analytica chimica acta  659  2010

      Show Abstract
      20103114 20103114
      Infectious delivery and expression of a 135 kb human FRDA genomic DNA locus complements Friedreich's ataxia deficiency in human cells.
      Gomez-Sebastian, Silvia, et al.
      Mol. Ther., 15: 248-54 (2007)  2007

      Show Abstract
      Human17235301 17235301
      Extra-mitochondrial localisation of frataxin and its association with IscU1 during enterocyte-like differentiation of the human colon adenocarcinoma cell line Caco-2
      Acquaviva, Fabio, et al
      J Cell Sci, 118:3917-24 (2005)  2005

      16091420 16091420
      Friedreich's ataxia, no changes in mitochondrial labile iron in human lymphoblasts and fibroblasts: a decrease in antioxidative capacity?
      Sturm, Brigitte, et al.
      J. Biol. Chem., 280: 6701-8 (2005)  2005

      Show Abstract
      15615730 15615730
      Erythroid differentiation and protoporphyrin IX down-regulate frataxin expression in Friend cells: characterization of frataxin expression compared to molecules involved in iron metabolism and hemoglobinization
      Becker, Erika M, et al
      Blood, 99:3813-22 (2002)  2002

      11986241 11986241
      Manganese superoxide dismutase induction by iron is impaired in Friedreich ataxia cells.
      Jiralerspong, S, et al.
      FEBS Lett., 509: 101-5 (2001)  2001

      Show Abstract
      11734214 11734214
      Frataxin deficiency enhances apoptosis in cells differentiating into neuroectoderm
      Santos, MM et al
      Hum Mole Genetics, 10(8):1935-1944 (2001)  2001

      11555630 11555630
      Maturation of wild-type and mutated frataxin by the mitochondrial processing peptidase.
      Koutnikova, H, et al.
      Hum. Mol. Genet., 7: 1485-9 (1998)  1998

      Show Abstract
      Immunoblotting (Western)9700204 9700204

      Data Sheet

      Title
      Anti-Frataxin, exon 4, clone 1G2 - Data Sheet