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    Rabbit Anti-Glutaredoxin 5/BF350 Conjugated antibody (bs-13395R-BF350)
    訂購熱線(xiàn):400-901-9800
    訂購郵箱:sales@bioss.com.cn
    訂購QQ:  400-901-9800
    技術(shù)支持:techsupport@bioss.com.cn
    說(shuō) 明 書(shū): 100ul  
    100ul/2980.00元
    大包裝/詢(xún)價(jià)
    產(chǎn)品編號 bs-13395R-BF350
    英文名稱(chēng) Rabbit Anti-Glutaredoxin 5/BF350 Conjugated antibody
    中文名稱(chēng) BF350標記的谷氧還蛋白5抗體
    別    名 C14orf87; Chromosome 14 open reading frame 87; FLB4739; GLRX 5; Glrx5; GLRX5_HUMAN; Glutaredoxin 5 homolog; Glutaredoxin related protein 5; Glutaredoxin-related protein 5; Glutaredoxin5; GRX5; MGC14129; mitochondrial; Monothiol glutaredoxin-5; PRO1238.  
    規格價(jià)格 100ul/2980元 購買(mǎi)        大包裝/詢(xún)價(jià)
    說(shuō) 明 書(shū) 100ul  
    研究領(lǐng)域 腫瘤  細胞生物  信號轉導  新陳代謝  線(xiàn)粒體  
    抗體來(lái)源 Rabbit
    克隆類(lèi)型 Polyclonal
    交叉反應 (predicted: Human, Mouse, Rat, Pig, Cow, Horse, Rabbit, Sheep, )
    產(chǎn)品應用 ICC=1:50-200 IF=1:50-200 
    not yet tested in other applications.
    optimal dilutions/concentrations should be determined by the end user.
    分 子 量 14kDa
    性    狀 Lyophilized or Liquid
    濃    度 1mg/ml
    免 疫 原 KLH conjugated synthetic peptide derived from human Glutaredoxin 5
    亞    型 IgG
    純化方法 affinity purified by Protein A
    儲 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol
    保存條件 Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.
    產(chǎn)品介紹 background:
    This gene encodes a mitochondrial protein, which is evolutionarily conserved. It is involved in the biogenesis of iron-sulfur clusters, which are required for normal iron homeostasis. Mutations in this gene are associated with autosomal recessive pyridoxine-refractory sideroblastic anemia. [provided by RefSeq, May 2010]

    Function:
    Monothiol glutaredoxin involved in the biogenesis of iron-sulfur clusters. Required for normal iron homeostasis. Required for normal regulation of hemoglobin synthesis by the iron-sulfur protein ACO1.

    Subunit:
    Homodimer.

    Subcellular Location:
    Mitochondrion.

    DISEASE:
    Defects in GLRX5 are a cause of anemia sideroblastic pyridoxine-refractory autosomal recessive (PRARSA) [MIM:205950]. A form of sideroblastic anemia not responsive to pyridoxine. Sideroblastic anemia is characterized by anemia of varying severity, hypochromic peripheral erythrocytes, systemic iron overload secondary to chronic ineffective erythropoiesis, and the presence of bone marrow ringed sideroblasts. Sideroblasts are characterized by iron-loaded mitochondria clustered around the nucleus.

    Similarity:
    Belongs to the glutaredoxin family. Monothiol subfamily.
    Contains 1 glutaredoxin domain.

    Database links:

    Entrez Gene: 51218 Human

    Entrez Gene: 73046 Mouse

    Entrez Gene: 362776 Rat

    Omim: 609588 Human

    SwissProt: Q86SX6 Human

    SwissProt: Q80Y14 Mouse

    Unigene: 532683 Human

    Unigene: 728210 Human

    Unigene: 29128 Mouse

    Unigene: 104008 Rat



    Important Note:
    This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
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