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merck millipore,默克密理博,MAB1594,Anti-Frataxin Antibody, exon 4, clone 1G2
产品名称：Anti-Frataxin Antibody, exon 4, clone 1G2
产品型号：MAB1594
Detect Frataxin using this Anti-Frataxin Antibody, exon 4, clone 1G2 validated for use in ELISA, IC, IF & WB.
merck millipore,默克密理博,MAB1594,Anti-Frataxin Antibody, exon 4, clone 1G2
- 产品介绍
重要规格表
品种反应性 主要应用 宿主 格式 抗体类型 R, H, M WB, ELISA, ICC M Ascites Monoclonal Antibody 描述 产品目录编号 MAB1594 品牌系列 Chemicon®
商名 - Chemicon
描述 Anti-Frataxin Antibody, exon 4, clone 1G2 Alternate Names - Friedreich ataxia protein
- Friedreich ataxia
背景信息 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). 产品信息 格式 Ascites 控制 - Liver, heart or skeletal muscle.
演示 Mouse monoclonal ascites IgG1κ in buffer containing liquid with no preservatives. 应用 应用 Detect Frataxin using this Anti-Frataxin Antibody, exon 4, clone 1G2 validated for use in ELISA, IC, IF & WB. 主要应用 - Western Blotting
- ELISA
- Immunocytochemistry
应用说明 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.生物信息 免疫原品种 Full length human Frataxin fused to TrpE. 表位 exon 4 克隆 1G2 浓缩 Please refer to the Certificate of Analysis for the lot-specific concentration. 宿主 Mouse 特异性 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). 同种型 IgG1κ 品种反应性 RatHumanMouse Species Reactivity Note Mouse and rat. Expected to cross-react with human. 抗体类型 Monoclonal Antibody Entrez基因编号 - NM_000144.3
Entrez基因汇总 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. 基因符号 - X25
- FXN
- FRDA
- FARR
- FA
- Fxn
- MGC57199
- CyaY
纯化方法 Unpurified UniProt编号 - Q16595
UniProt汇总 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产品使用声明 质量保证 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.使用声明 - 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.
储存和货运信息 存储条件 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包装信息 数量 100 µL
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