Human HBA2 Recombinant Protein (RPPB3661)

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SKU:
RPPB3661
Product type:
Recombinant Protein
Size:
20ug
Species:
Human
Target:
HBA2
Synonyms:
Hemoglobin alpha chain
Synonyms:
hemoglobin alpha 2
Synonyms:
hemoglobin subunit alpha
Synonyms:
Alpha-globin
Source:
Escherichia Coli
Uniprot:
P69905
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Description

Product Name:Human HBA2 Recombinant Protein
Product Code:RPPB3661
Size:20µg
Species:Human
Target:HBA2
Synonyms:Hemoglobin alpha chain, hemoglobin alpha 2, hemoglobin subunit alpha, Alpha-globin, alpha-2 globin, HBH.
Source:Escherichia Coli
Physical Appearance:Sterile Filtered colorless solution.
Formulation:The HBA2 solution (1mg/1ml) contains 20mM Tris-HCl buffer (pH 8.0), 100mM NaCl, 2mM DTT, 2M urea and 20% glycerol.
Stability:Store at 4°C if entire vial will be used within 2-4 weeks. Store, frozen at -20°C for longer periods of time. For long term storage it is recommended to add a carrier protein (0.1% HSA or BSA).Avoid multiple freeze-thaw cycles.
Purity:Greater than 90% as determined by SDS-PAGE.
Amino Acid Sequence:MRGSHHHHHH GMASMTGGQQ MGRDLYDDDD KDRWGSHMVL SPADKTNVKA AWGKVGAHAG EYGAEALERM FLSFPTTKTY FPHFDLSHGS AQVKGHGKKV ADALTNAVAH VDDMPNALSA LSDLHAHKLR VDPVNFKLLS HCLLVTLAAH LPAEFTPAVH ASLDKFLASV STVLTSKYR

HBA2 is a member of the globin family. HBA2 takes part in oxygen transport from the lung to the different peripheral tissues. The coding sequences of HBA2 and HBA1 are equal but have a small difference over the 5' untranslated regions and the introns, and a large difference over the 3' untranslated regions. HbA is constituted from two alpha chains and two beta chains which in standard adult life hold about 97% of the total hemoglobin; alpha chains associate with delta chains to create HbA-2 that with HbF (fetal hemoglobin) form the remaining 3% of adult hemoglobin. Alpha thalassemia is caused by removal of each of the alpha genes or removal of both HBA2 and HBA1; nondeletion alpha thalassemias has also been reported.

HBA2 Human Recombinant produced in E. coli is a single polypeptide chain containing 179 amino acids (1-142) and having a molecular mass of 19.5 kDa.HBA2 is fused to a 37 amino acid His-tag at N-terminus & purified by proprietary chromatographic techniques.

UniProt Protein Function:HBA1: Involved in oxygen transport from the lung to the various peripheral tissues. Defects in HBA1 may be a cause of Heinz body anemias (HEIBAN). 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. Defects in HBA1 are the cause of alpha-thalassemia (A- THAL). The thalassemias are the most common monogenic diseases and occur mostly in Mediterranean and Southeast Asian populations. The hallmark of alpha-thalassemia is an imbalance in globin-chain production in the adult HbA molecule. The level of alpha chain production can range from none to very nearly normal levels. Deletion of both copies of each of the two alpha-globin genes causes alpha(0)-thalassemia, also known as homozygous alpha thalassemia. Due to the complete absence of alpha chains, the predominant fetal hemoglobin is a tetramer of gamma-chains (Bart hemoglobin) that has essentially no oxygen carrying capacity. This causes oxygen starvation in the fetal tissues leading to prenatal lethality or early neonatal death. The loss of three alpha genes results in high levels of a tetramer of four beta chains (hemoglobin H), causing a severe and life-threatening anemia known as hemoglobin H disease. Untreated, most patients die in childhood or early adolescence. The loss of two alpha genes results in mild alpha-thalassemia, also known as heterozygous alpha-thalassemia. Affected individuals have small red cells and a mild anemia (microcytosis). If three of the four alpha-globin genes are functional, individuals are completely asymptomatic. Some rare forms of alpha-thalassemia are due to point mutations (non- deletional alpha-thalassemia). The thalassemic phenotype is due to unstable globin alpha chains that are rapidly catabolized prior to formation of the alpha-beta heterotetramers. Alpha(0)-thalassemia is associated with non-immune hydrops fetalis, a generalized edema of the fetus with fluid accumulation in the body cavities due to non-immune causes. Non- immune hydrops fetalis is not a diagnosis in itself but a symptom, a feature of many genetic disorders, and the end-stage of a wide variety of disorders. Defects in HBA1 are the cause of hemoglobin H disease (HBH). HBH is a form of alpha-thalassemia due to the loss of three alpha genes. This results in high levels of a tetramer of four beta chains (hemoglobin H), causing a severe and life-threatening anemia. Untreated, most patients die in childhood or early adolescence. Belongs to the globin family.
UniProt Protein Details:

Protein type:Carrier

Chromosomal Location of Human Ortholog: 16p13.3

Cellular Component: membrane; hemoglobin complex; extracellular region; cytosol

Molecular Function:haptoglobin binding; protein binding; peroxidase activity; iron ion binding; heme binding; oxygen binding; oxygen transporter activity

Biological Process: receptor-mediated endocytosis; response to hydrogen peroxide; hydrogen peroxide catabolic process; protein heterooligomerization; bicarbonate transport; oxygen transport

Disease: Hemoglobin H Disease; Heinz Body Anemias; Alpha-thalassemia

NCBI Summary:The 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]
UniProt Code:P69905
NCBI GenInfo Identifier:57013850
NCBI Gene ID:3040
NCBI Accession:P69905.2
UniProt Secondary Accession:P69905,P01922, Q1HDT5, Q3MIF5, Q53F97, Q96KF1, Q9NYR7 Q9UCM0,
UniProt Related Accession:P69905
Molecular Weight:15,258 Da
NCBI Full Name:Hemoglobin subunit alpha
NCBI Synonym Full Names:hemoglobin, alpha 2
NCBI Official Symbol:HBA2  
NCBI Official Synonym Symbols:HBH; HBA-T2  
NCBI Protein Information:hemoglobin subunit alpha; alpha globin; alpha-globin; alpha-2 globin; hemoglobin alpha chain
UniProt Protein Name:Hemoglobin subunit alpha
UniProt Synonym Protein Names:Alpha-globin; Hemoglobin alpha chain
Protein Family:Hemoglobin
UniProt Gene Name:HBA1  
UniProt Entry Name:HBA_HUMAN
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