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- 适应物种:
Human,
- 应用范围:
WB,
- 抗体英文名:
Hemoglobin Beta
- 规格:
100ul
英文名称Hemoglobin Beta中文名称血红蛋白β抗体别 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 研究领域心血管 细胞生物 抗体来源Rabbit克隆类型Polyclonal交叉反应Human, 产品应用WB=1:500-2000 ELISA=1:5000-10000
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.分 子 量15.5kDa细胞定位细胞浆 分泌型蛋白 性 状Liquid浓 度1mg/ml免 疫 原KLH conjugated synthetic peptide derived from human Hemoglobin beta:51-147/147 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Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.PubMedPubMed产品介绍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.
SWISS:
P68871
Gene ID:
3043
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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文献和实验氧合血红蛋白 oxyhemoglobin 血红蛋白和氧分子的结合物。为通过与氧可逆性地结合而生成。血红蛋白就是以这种形式运输氧气。血红素铁原子为二价与 O2 、卟啉的 4个 N,与珠蛋白的组氨酸残基(咪唑)结合,成为八面体的络合物结构。它与游离的血红蛋白不同,是低自旋( spin)物质,吸收光谱也类似细胞色素还原型。氧分压和氧合血红蛋白的生成百分率( %)的图即结合曲线(解离曲线),由于血红素间的相互作用的变构( alloste- ric)效应而呈 S型。氧合血红蛋白
红细胞中运输氧及二氧化碳的蛋白质,由球蛋白与血红素结合而成。分子量约 64, 500,其中蛋白质占 96%。血红素由二价铁原卟啉组成。球蛋白由两对不同肽链组成。每个肽链含 1个血红素分子,所以 1个血红蛋白分子能结合 4个氧分子。正常人血红蛋白( Hb)含血红蛋白 A( HbA)、血红蛋白 A2 ( HbA2 )及血红蛋白 F( HbF)。 HbA由一对α链及一对β链构成(α 2 β 2 ),约占成人血红蛋白的 97%。 HbA2 由α链及δ链组成(α 2 δ
人 血红蛋白 ( Hb ) 酶联免疫分析(ELISA ) 试剂盒使用说明书 本试剂仅供研究使用 目的:本试剂盒用于测定人血清,血浆及相关液体样本中血红蛋白(Hb) 含量。 实验原理: 本试剂盒应用双抗体夹心法测定标本中人血红蛋白(Hb) 水平。用纯化的人血红蛋白(Hb) 抗体包被微孔板,制成固相抗体,往包被单抗的微孔中依次加入血红蛋白(Hb) ,再与HRP 标记的血红蛋白(Hb) 抗体结合,形成抗体- 抗原- 酶标
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