货号：MAB1594

品牌：Merck Millipore

规格：100UL

目录价：￥6742.00

市场价格：￥5730.70

会员价格：￥5393.60

金山科研平台，产品价格货期咨询微信：jinshanbio Description: Anti-Frataxin Antibody, exon 4, clone 1G2 | MAB1594 View All» Promotional Text: Special Shipping Offer on Antibodies100% Performance Guaranteed View All» Trade Name: Chemicon (Millipore) View All» Specificity: Human 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). View All» Molecular Weight: ~ 18 kDa View All» Epitope: exon 4 View All» Immunogen: Full length human Frataxin fused to TrpE. View All» Clone: 1G2 View All» Isotype: IgG1κ View All» Background Information: Frataxin 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). View All» Species Reactivity:

• Human

• Mouse

• Rat
  View All» Species Reactivity Note: Mouse and rat. Expected to cross-react with human. View All» Application Notes: Immunofluorescence: 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. View All» Control: Liver, heart or skeletal muscle. View All» Quality Assurance: Routinely evaluated by Western Blot on PC12 lysates.Western Blot Analysis: 1:1000 dilution of this lot detected Frataxin on 10 μg of PC12 lysates. View All» Purification Method: Unpurified View All» Presentation: Mouse monoclonal ascites IgG1κ in buffer containing liquid with no preservatives. View All» Storage Conditions: Stable 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 View All» UniProt Number: Q16595 View All» Entrez Gene Number: NM_000144.3 View All» Gene Symbol:
  • FXN

  • FRDA

  • FARR

  • FA

  • Fxn

  • MGC57199

  • CyaY

  • X25
    View All» Alternate Names:
    • Friedreich ataxia

    • Friedreich ataxia protein
      View All» 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. View All» Key Applications:
      • ELISA

      • Immunocytochemistry

      • Immunofluorescence

      • Western Blotting
        View All» Entrez Gene 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 occurs at this locus and two transcript variants encoding distinct isoforms have been identified. View All» UniProt Summary: FUNCTION: 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 View All» Brand Family: Chemicon View All» Product Name: Anti-Frataxin Antibody, exon 4, clone 1G2 | MAB1594 View All» Concentration: Variable View All» Antibody Type: Monoclonal Antibody View All» Qty/Pk: 100 µL View All» Format: Ascites View All» Host: Mouse View All»
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