FITC标记的血红蛋白α1/α-Globin抗体-抗体-抗体-生物在线
上海沪震实业有限公司
FITC标记的血红蛋白α1/α-Globin抗体

FITC标记的血红蛋白α1/α-Globin抗体

商家询价

产品名称: FITC标记的血红蛋白α1/α-Globin抗体

英文名称: Anti-HBA1/FITC

产品编号: HZ-

产品价格: null

产品产地: 中国/上海

品牌商标: HZbscience

更新时间: 2023-08-17T10:24:20

使用范围: IF=1:50-200

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 Rabbit Anti-HBA1/FITC Conjugated antibody

FITC标记的血红蛋白α1/α-Globin抗体

 

英文名称 Anti-HBA1/FITC
中文名称 FITC标记的血红蛋白α1/α-Globin抗体
别    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.  
规格价格 100ul/2980元 购买        大包装/询价
说 明 书 100ul  
研究领域 细胞生物  免疫学  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 Human, Rat, Monkey, 
产品应用 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    状 Lyophilized or Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human HBA1
亚    型 IgG
纯化方法 affinity purified by Protein A
储 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存条件 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.
产品介绍 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

血红蛋白参与从肺部到各种外周组织的氧转运。α(HBA)和β(HBB)位点决定血红蛋白中2种多肽链的结构。正常血红蛋白四聚体由两个α链和两个β链组成。β-珠蛋白突变体导致镰状细胞贫血。β链缺失导致β地中海贫血。可检测到的β珠蛋白减少导致β加地中海贫血。参与氧气从肺部传输到各种外周组织。