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Rabbit Anti-Hemoglobin Beta/BF555 Conjugated antibody (bs-8554R-BF555)
訂購(gòu)熱線:400-901-9800
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說(shuō) 明 書(shū): 100ul  
100ul/2980.00元
大包裝/詢(xún)價(jià)
產(chǎn)品編號(hào) bs-8554R-BF555
英文名稱(chēng)1 Rabbit Anti-Hemoglobin Beta/BF555 Conjugated antibody
中文名稱(chēng) BF555標(biāo)記的血紅蛋白β抗體
別    名 Beta 1 globin; beta globin; beta globin chain; BETA GLOBIN TYPE; BETA THALASSEMIAS; CD113t C; CD113t-C; ERYTHREMIA; HBB; Hemoglobin beta 1 chain; hemoglobin beta chain; hemoglobin beta chain complex; Hemoglobin beta chain, major form; HEMOGLOBIN BETA LOCUS; METHEMOGLOBINEMIA  
規(guī)格價(jià)格 100ul/2980元 購(gòu)買(mǎi)        大包裝/詢(xún)價(jià)
說(shuō) 明 書(shū) 100ul  
研究領(lǐng)域 心血管  細(xì)胞生物  
抗體來(lái)源 Rabbit
克隆類(lèi)型 Polyclonal
交叉反應(yīng) Human, 
產(chǎn)品應(yīng)用 IF=1:50-200 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 15.5kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human Hemoglobin beta
亞    型 IgG
純化方法 affinity purified by Protein A
儲(chǔ) 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:
The alpha (HBA) and beta (HBB) loci determine the structure of the 2 types of polypeptide chains in adult hemoglobin, Hb A. 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. The order of the genes in the beta-globin cluster is 5'-epsilon -- gamma-G -- gamma-A -- delta -- beta--3'. [provided by RefSeq, Jul 2008].

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

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: 3043 Human

Omim: 141900 Human

SwissProt: P68871 Human

Unigene: 523443 Human



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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