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  4. GLRX5 Antibody, FITC conjugated

GLRX5 Antibody, FITC conjugated

  • 中文名稱：
    GLRX5兔多克隆抗體, FITC偶聯(lián)
  • 貨號：
    CSB-PA769767LC01HU
  • 規(guī)格：
    ￥880
  • 其他：

產(chǎn)品詳情

  • 產(chǎn)品名稱：
    Rabbit anti-Homo sapiens (Human) GLRX5 Polyclonal antibody
  • Uniprot No.：
  • 基因名：
    GLRX5
  • 別名：
    C14orf87 antibody; Chromosome 14 open reading frame 87 antibody; FLB4739 antibody; GLRX 5 antibody; Glrx5 antibody; GLRX5_HUMAN antibody; Glutaredoxin 5 homolog antibody; Glutaredoxin related protein 5 antibody; Glutaredoxin-related protein 5 antibody; Glutaredoxin5 antibody; GRX5 antibody; MGC14129 antibody; mitochondrial antibody; Monothiol glutaredoxin-5 antibody; PRO1238 antibody
  • 宿主：
    Rabbit
  • 反應(yīng)種屬：
    Human
  • 免疫原：
    Recombinant Human Glutaredoxin-related protein 5, mitochondrial protein (32-157AA)
  • 免疫原種屬：
    Homo sapiens (Human)
  • 標記方式：
    FITC
  • 克隆類型：
    Polyclonal
  • 抗體亞型：
    IgG
  • 純化方式：
    >95%, Protein G purified
  • 濃度：
    It differs from different batches. Please contact us to confirm it.
  • 保存緩沖液：
    Preservative: 0.03% Proclin 300
    Constituents: 50% Glycerol, 0.01M PBS, pH 7.4
  • 產(chǎn)品提供形式：
    Liquid
  • 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：
    Monothiol glutaredoxin involved in mitochondrial iron-sulfur (Fe/S) cluster transfer. Receives 2Fe/2S clusters from scaffold protein ISCU and mediates their transfer to apoproteins, to the 4Fe/FS cluster biosynthesis machinery, or export from mitochondrion. Required for normal regulation of hemoglobin synthesis by the iron-sulfur protein ACO1.
  • 基因功能參考文獻：
    1. GLRX5 rs1007814 showed a statistically marginally significant difference between cases and controls in genotype frequency (case/control: CC 1:6; CT 112:78; TT 752:505, P=0.049361), but no significant differences in allele distribution [odds ratio (OR)=0.852805]In men, we found a minor difference in the genotype frequency (case/control: CC 0:3; CT 72:36; TT 411:280, P=0.037370) and not in allele distribution (OR=1.142857) PMID: 27893590
    2. Patients with GLRX5-associated variant nonketotic hyperglycemia had normal development with childhood-onset spastic paraplegia, spinal lesion, and optic atrophy. PMID: 24334290
    3. crystal structure of GLRX5 revealed a tetrameric organization with the [2Fe-2S] clusters buried in the interior and shielded from the solvent by the conserved beta1-alpha2 loop PMID: 21029046
    4. Glutaredoxin 5 deficiency causes sideroblastic anemia by specifically impairing heme biosynthesis and depleting cytosolic iron in human erythroblasts PMID: 20364084
    5. No GLRX5 mutations were found among sixty CSA probands examined PMID: 19731322
    6. Mutations in GLRX5 is associated with sideroblastic-like microcytic anemia and iron overload PMID: 17485548

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  • 相關(guān)疾?。?/div>
    Anemia, sideroblastic, 3, pyridoxine-refractory (SIDBA3); Spasticity, childhood-onset, with hyperglycinemia (SPAHGC)
  • 亞細胞定位：
    Mitochondrion matrix.
  • 蛋白家族：
    Glutaredoxin family, Monothiol subfamily
  • 數(shù)據(jù)庫鏈接：

    HGNC: 20134

    OMIM: 609588

    KEGG: hsa:51218

    STRING: 9606.ENSP00000328570

    UniGene: Hs.744943