货号：AB15080

品牌：Merck Millipore

规格：100UL

目录价：￥5513.00

市场价格：￥4686.05

会员价格：￥4410.40

金山科研平台，产品价格货期咨询微信：jinshanbio Description: Anti-Frataxin Antibody | AB15080 View All» Promotional Text: Special Shipping Offer on Antibodies100% Performance Guaranteed View All» Specificity: Cat. # AB15080 recognizes Frataxin. View All» Molecular Weight: Approx. 18 kDa View All» Epitope: mature chain View All» Immunogen: GST-tagged Frataxin corresponding to mature chain. View All» Background Information: Frataxin 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). View All» Species Reactivity:

• Human

• Mouse

• Rat
  View All» Species Reactivity Note: Human, Mouse and Rat. View All» Control: Human heart lysate. View All» Quality Assurance: Routinely 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 View All» Purification Method: GST-depleted sera. View All» Presentation: Rabbit polyclonal serum in buffer containing 0.05% NaN3. View All» Storage Conditions: Stable for 1 year at -20ºC from date of receipt. View All» UniProt Number: Q16595 View All» Entrez Gene Number: NP_000135 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: 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. [provided by RefSeq] View All» UniProt Summary: Function: Probably involved in iron homeostasis. Anti-apoptotic protein which prevents mitochondrial damage and reactive oxygen species (ROS) production. Ref.13Subunit structure: Monomer.Subcellular location: Mitochondrion. Note= Mitochondrial and extramitochondrial. Ref.13 Ref.8 Ref.9Tissue specificity: Frataxin(81-210) is expressed in the heart, peripheral blood lymphocytes and dermal fibroblasts. Ref.4Post-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.21Sequence similarities: Belongs to the frataxin family. View All» Product Name: Anti-Frataxin View All» Antibody Type: Polyclonal Antibody View All» Qty/Pk: 100 µL View All» Format: Purified View All» Host: Rabbit View All»
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