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  4. FARS2 Antibody

FARS2 Antibody

  • 中文名稱：
    FARS2兔多克隆抗體
  • 貨號：
    CSB-PA008429GA01HU
  • 規(guī)格：
    ￥3,900
  • 其他：

產(chǎn)品詳情

  • Uniprot No.：
  • 基因名：
    FARS2
  • 別名：
    dJ236A3.1 (phenylalanine tRNA synthetase) antibody; dJ520B18.2 (FARS1 (phenylalanine tRNA synthetase)) antibody; FARS1 antibody; Fars2 antibody; HSPC320 antibody; Phenylalanine translase antibody; Phenylalanine tRNA ligase 2; mitochondrial antibody; Phenylalanine tRNA ligase antibody; Phenylalanine tRNA synthetase 1 (mitochondrial) antibody; Phenylalanine tRNA synthetase 2 (mitochondrial) antibody; Phenylalanine--tRNA ligase antibody; Phenylalanyl tRNA synthetase 2 antibody; Phenylalanyl-tRNA synthetase; mitochondrial antibody; PheRS antibody; SYFM_HUMAN antibody
  • 宿主：
    Rabbit
  • 反應種屬：
    Human,Mouse,Rat
  • 免疫原：
    Human FARS2
  • 免疫原種屬：
    Homo sapiens (Human)
  • 抗體亞型：
    IgG
  • 純化方式：
    Antigen Affinity purified
  • 濃度：
    It differs from different batches. Please contact us to confirm it.
  • 保存緩沖液：
    PBS with 0.1% Sodium Azide, 50% Glycerol, pH 7.3. -20°C, Avoid freeze / thaw cycles.
  • 產(chǎn)品提供形式：
    Liquid
  • 應用范圍：
    ELISA,WB
  • Protocols：
  • 儲存條件：
    Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
  • 貨期：
    Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.
  • 用途：
    For Research Use Only. Not for use in diagnostic or therapeutic procedures.

產(chǎn)品評價

靶點詳情

  • 功能：
    Is responsible for the charging of tRNA(Phe) with phenylalanine in mitochondrial translation. To a lesser extent, also catalyzes direct attachment of m-Tyr (an oxidized version of Phe) to tRNA(Phe), thereby opening the way for delivery of the misacylated tRNA to the ribosome and incorporation of ROS-damaged amino acid into proteins.
  • 基因功能參考文獻：
    1. Based on the phenotypic data of previously reported subjects and the two subjects reported here, we conclude that FARS2 deficiency can be associated with two phenotypes: (i) an epileptic phenotype, and (ii) a spastic paraplegia phenotype. PMID: 29126765
    2. in patients with drug-resistant infantile spasm syndrome, associated with focal seizures, mild metabolic changes, and cerebral atrophy with volume loss of white matter on MRI, mutations in FARS2 should be considered. PMID: 27549011
    3. Kinetic and structural changes in HsmtPheRS, induced by pathogenic mutations in human FARS2 have been described. PMID: 28419689
    4. A newly identified missense mutation in FARS2 causes autosomal-recessive spastic paraplegia. PMID: 26553276
    5. this study expands the phenotypic spectrum of FARS2 related disease and emphasizes intragenic deletion in the list of causative mutations. PMID: 25851414
    6. the three FARS2 mutations directly impair aminoacylation function and stability of mtPheRS, leading to a decrease in overall tRNA charging capacity. PMID: 22833457
    7. Two phenylalanyl-tRNA synthetase variants Ser57Cys and Asp280Ser both display wild-type aminoacylation activity and stability with respect to their free energies of unfolding, but are less stable at low hydrogen-ion concentration (pH). PMID: 21601574
    8. The recombinant human enzyme has been purified to homogeneity and crystallized in complex with phenylalanine and ATP. PMID: 17768348
    9. Formation of the PheRS-tRNAPhe complex in human mitochondria must be accompanied by considerable rearrangement of the anticodon binding domain upon tRNA binding. PMID: 18611382
    10. Mitochondrial and cytoplasmic phenylalanyl-tRNA synthetases (HsmtPheRS and HsctPheRS, respectively) catalyze direct attachment of m-Tyr to tRNA(Phe). PMID: 19549855
    11. these results indicate that conformational flexibility of the two functional modules in mtPheRS is essential for its phenylalanylation activity. PMID: 19737557

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  • 相關(guān)疾?。?/div>
    Combined oxidative phosphorylation deficiency 14 (COXPD14); Spastic paraplegia 77, autosomal recessive (SPG77)
  • 亞細胞定位：
    Mitochondrion matrix. Mitochondrion.
  • 蛋白家族：
    Class-II aminoacyl-tRNA synthetase family
  • 數(shù)據(jù)庫鏈接：

    HGNC: 21062

    OMIM: 611592

    KEGG: hsa:10667

    STRING: 9606.ENSP00000274680

    UniGene: Hs.484547