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血红蛋白α1/α-Globin抗体
产品名称:
血红蛋白α1/α-Globin抗体
英文名称:
HBA1
产品类别:
抗体
产品编号:
Ys-0847R
产品应用:
WB ELISA IHC-P IHC-F IF
性状:
Liquid
纯化方法:
affinity purified by Protein A
保质期:
12个月
保存条件:
Shipped at 4℃. Store at -20 °C for one year. Avoid
[价格]
规格 价格 库存
50ul ¥ 1200 7
100ul ¥ 1900 6
200ul ¥ 2900 3

产品详情

 

产品编号 Ys-0847R
英文名称 HBA1
中文名称 血红蛋白α1/α-Globin抗体
别    名 Alpha 1 globin; Alpha globin; Alpha one globin; Alpha-globin; HBA_HUMAN; HBA2; Hemoglobin alpha 1; Hemoglobin alpha 1 chain; Hemoglobin alpha 1 globin chain; Hemoglobin alpha 2; Hemoglobin alpha chain; Hemoglobin subunit alpha; MGC126895; MGC12689.  
研究领域 心血管  细胞类型标志物  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 Human,  (predicted: Rat, Monkey, )
产品应用 WB=1:500-2000 ELISA=1:5000-10000 IHC-P=1:100-500 IHC-F=1:100-500 IF=1:100-500 (石蜡切片需做抗原修复)
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理论分子量 15kDa
细胞定位 细胞膜 
性    状 Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human HBA1: 56-141/141 
亚    型 IgG
纯化方法 affinity purified by Protein A
缓 冲 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存条件 Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
注意事项 This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed PubMed
产品介绍 he human alpha globin gene cluster located on chromosome 16 spans about 30 kb and includes seven loci: 5'- zeta - pseudozeta - mu - pseudoalpha-1 - alpha-2 - alpha-1 - theta - 3'. The alpha-2 (HBA2) and alpha-1 (HBA1) coding sequences are identical. These genes differ slightly over the 5' untranslated regions and the introns, but they differ significantly over the 3' untranslated regions. Two alpha chains plus two beta chains constitute HbA, which in normal adult life comprises about 97% of the total hemoglobin; alpha chains combine with delta chains to constitute HbA-2, which with HbF (fetal hemoglobin) makes up the remaining 3% of adult hemoglobin. Alpha thalassemias result from deletions of each of the alpha genes as well as deletions of both HBA2 and HBA1; some nondeletion alpha thalassemias have also been reported. [provided by RefSeq, Jul 2008]

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.

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