Human Hemoglobin H ELISA Kit | HBH elisa kit

Human Hemoglobin H ELISA Kit

Cat. Num. AAA8935

• Gene Names: HBA2; HBH; HBA-T2
• Reactivity: Human
• Synonyms: Hemoglobin H; Human Hemoglobin H ELISA Kit; HBH elisa kit

• Reactivity: Human
• Sequence Length: 142

• Sensitivity: 5.0 ug/ml.
• Preparation and Storage: Store all reagents at 2-8 degree C

Typical Testing Data/Standard Curve (for reference only)

Related Product Information for HBH elisa kit

INTRODUCTION ELISA is a simple and highly sensitive method of analysis that allows for simultaneous and rapid quantification of a large number of samples. The assay is based on the specific recognition of the target compound (analyte/antigen) by antibodies which bind to the compound. The antigen-antibody complex is detected and measured with the aid of an enzyme-labeled antibody or antigen. Upon addition of a non-colored reagent, the enzyme produces a color reaction where the color intensity is directly or inversely proportional to the concentration of the analyte in the sample.

INTENDED USE This ELISA kit is intended for laboratory in vitro research use only, not for drug, household or other use! The Stop Solution changes the color from blue to yellow and the intensity of the color is measured at 450 nm by a spectrophotometer. In order to measure theconcentration of Human HBH in the sample, this ELISA kit includes a set of calibration standards. The calibration standards are assayed at the same time as the samples and allow the operator to produce a standard curve of Optical Density versus Human HBH concentration. The concentration of Human HBH in the samples is then determined by comparing the O.D. of the samples to the standard curve.

NCBI and Uniprot Product Information

• NCBI GI #: 57013850
• NCBI GeneID: 3040
• UniProt Accession #: P69905; P01922; Q1HDT5; Q3MIF5; Q53F97; Q96KF1; Q9NYR7; Q9UCM0
• Molecular Weight: 15,258 Da
• NCBI Official Full Name: Hemoglobin subunit alpha
• NCBI Official 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
• Protein Family: Hemoglobin
• UniProt Gene Name: HBA1
• UniProt Entry Name: HBA_HUMAN

NCBI Description

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 Description

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.

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; oxygen transport; bicarbonate transport; protein heterooligomerization; hydrogen peroxide catabolic process

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