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    Rabbit Anti-HBA1/BF594 Conjugated antibody (bs-0847R-BF594)
    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-0847R-BF594
    英文名稱(chēng) Rabbit Anti-HBA1/BF594 Conjugated antibody
    中文名稱(chēng) BF594標記的血紅蛋白α1/α-Globin抗體
    別    名 Alpha 1 globin; Alpha globin; Alpha one globin; Alpha-globin; HBA_HUMAN; HBA1; HBA2; Hemoglobin alpha 1; Hemoglobin alpha 1 chain; Hemoglobin alpha 1 globin chain; Hemoglobin alpha 2; Hemoglobin alpha chain; Hemoglobin subunit alpha; MGC126895; MGC12689.  
    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 細胞生物  免疫學(xué)  
    抗體來(lái)源 Rabbit
    克隆類(lèi)型 Polyclonal
    交叉反應
    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 15kDa
    性    狀 Lyophilized or Liquid
    濃    度 1mg/ml
    免 疫 原 KLH conjugated synthetic peptide derived from human HBA1
    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:
    Hemoglobin is involved in oxygen transport from the lung to the various peripheral tissues. The alpha (HBA) and beta (HBB) loci determine the structure of the 2 types of polypeptide chains in adult Hemoglobin. The normal adult Hemoglobin tetramer consists of two alpha chains and two beta chains. Mutant beta globin causes sickle cell anemia. Absence of beta chain causes beta zero thalassemia. Reduced amounts of detectable beta globin causes beta plus thalassemia.Involved in oxygen transport from the lung to the various peripheral tissues.

    Function:
    Involved in oxygen transport from the lung to the various peripheral tissues.

    Subunit:
    Heterotetramer of two alpha chains and two beta chains in adult hemoglobin A (HbA); two alpha chains and two delta chains in adult hemoglobin A2 (HbA2); two alpha chains and two epsilon chains in early embryonic hemoglobin Gower-2; two alpha chains and two gamma chains in fetal hemoglobin F (HbF).

    Tissue Specificity:
    Red blood cells.

    Post-translational modifications:
    The initiator Met is not cleaved in variant Thionville and is acetylated.

    DISEASE:
    Defects in HBA1/HBA2 may be a cause of Heinz body anemias (HEIBAN) [MIM:140700]. This is a form of non-spherocytic hemolytic anemia of Dacie type 1. After splenectomy, which has little benefit, basophilic inclusions called Heinz bodies are demonstrable in the erythrocytes. Before splenectomy, diffuse or punctate basophilia may be evident. Most of these cases are probably instances of hemoglobinopathy. The hemoglobin demonstrates heat lability. Heinz bodies are observed also with the Ivemark syndrome (asplenia with cardiovascular anomalies) and with glutathione peroxidase deficiency.

    Similarity:
    Belongs to the globin family.

    Database links:

    Entrez Gene: 3039 Human

    Entrez Gene: 3040 Human

    Omim: 141800 Human

    Omim: 141850 Human

    SwissProt: P69905 Human

    SwissProt: P01942 Mouse

    SwissProt: P01946 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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