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Rabbit Anti-Glutaredoxin 5/BF594 Conjugated antibody (bs-13395R-BF594)
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說 明 書: 100ul  
100ul/2980.00元
大包裝/詢價
產品編號 bs-13395R-BF594
英文名稱 Rabbit Anti-Glutaredoxin 5/BF594 Conjugated antibody
中文名稱 BF594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.  
規格價格 100ul/2980元 購買        大包裝/詢價
說 明 書 100ul  
研究領域 腫瘤  細胞生物  信號轉導  新陳代謝  線粒體  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應
產品應用 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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