AHSP Human

Alpha Hemoglobin Stabilizing Protein Human Recombinant
Cat. No.
BT19696
Source
Escherichia Coli.
Synonyms
Alpha-hemoglobin-stabilizing protein, Erythroid-associated factor, Erythroid differentiation-related factor, AHSP, EDRF, ERAF.
Appearance
Sterile Filtered colorless solution.
Purity
Greater than 95.0% as determined by SDS-PAGE.
Usage
THE BioTek's products are furnished for LABORATORY RESEARCH USE ONLY. The product may not be used as drugs, agricultural or pesticidal products, food additives or household chemicals.
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Description

AHSP Human Recombinant produced in E.Coli is a single, non-glycosylated, polypeptide chain containing 102 amino acids (1-102 a.a.) and having a molecular mass of 11.8kDa.
The AHSP is purified by proprietary chromatographic techniques.

Product Specs

Introduction
Alpha-hemoglobin stabilizing protein (AHSP) is a protein found specifically in red blood cells. It acts as a helper molecule, preventing the clumping of a component of hemoglobin called A-hemoglobin during the normal development of red blood cells. AHSP specifically safeguards free A-hemoglobin from forming clumps within living cells and in solutions. It is believed that AHSP plays a role in managing conditions where there is an excess of alpha-hemoglobin, such as beta-thalassemia. Moreover, AHSP contributes to the stability of the alpha-globin chain during the formation of red blood cells in humans. AHSP achieves this by stabilizing the alpha-Hb chain, thus preventing its clumping and the subsequent generation of harmful molecules called ROS, which are known to cause cell death. AHSP is found in both blood and bone marrow. Existing as a single unit, it can pair with a free alpha-hemoglobin molecule to form a two-part unit. However, AHSP does not bind to beta-hemoglobin or to the complete hemoglobin A molecule. Interestingly, AHSP levels are reduced in transmissible spongiform encephalopathies (TSEs), a group of prion diseases affecting the brain.
Description
AHSP Human Recombinant, produced in E. coli bacteria, is a single-chain protein that is not modified by the addition of sugar molecules. It consists of 102 amino acids (specifically, amino acids 1 through 102) and has a molecular weight of 11.8 kDa. The purification of AHSP is achieved through specialized chromatographic methods.
Physical Appearance
A clear, colorless solution that has been sterilized by filtration.
Formulation
The AHSP protein solution (containing 1 mg of AHSP per ml of solution) is prepared in a solution containing 20 mM Tris-HCl buffer at a pH of 8.0 and 10% glycerol.
Stability
For optimal storage, keep at 4°C if the entire vial will be used within 2-4 weeks. For extended storage, freeze at -20°C. When storing long-term, it is advisable to add a carrier protein (0.1% HSA or BSA). Repeated freezing and thawing should be avoided.
Purity
The purity of the protein is greater than 95.0% as determined by SDS-PAGE analysis.
Synonyms
Alpha-hemoglobin-stabilizing protein, Erythroid-associated factor, Erythroid differentiation-related factor, AHSP, EDRF, ERAF.
Source
Escherichia Coli.
Amino Acid Sequence
MALLKANKDL ISAGLKEFSV LLNQQVFNDP LVSEEDMVTV VEDWMNFYIN YYRQQVTGEP QERDKALQEL RQELNTLANP FLAKYRDFLK SHELPSHPPP SS.

Product Science Overview

Function and Mechanism

AHSP specifically binds to free alpha-globin monomers, preventing their harmful aggregation and precipitation . During normal erythroid cell development, AHSP acts as a chaperone, ensuring that alpha-globin is safely transferred to beta-globin to form a stable heterodimer. This heterodimer then combines with another heterodimer to form the tetrameric hemoglobin, which is essential for oxygen transport in the blood .

In conditions where there is an excess of alpha-globin, such as in beta-thalassemia, AHSP plays a protective role by binding to the free alpha-globin and preventing its aggregation. This modulation helps in reducing the severity of diseases associated with alpha-globin excess .

Clinical Significance

The role of AHSP is particularly significant in the context of beta-thalassemia, a genetic disorder characterized by reduced or absent beta-globin production. In such cases, the excess alpha-globin can lead to severe clinical symptoms due to its tendency to aggregate and precipitate. AHSP helps mitigate these effects by binding to the free alpha-globin, thus acting as a potential modulatory agent in the treatment of beta-thalassemia .

Recombinant AHSP

Recombinant AHSP refers to the protein produced through recombinant DNA technology, which allows for the production of large quantities of the protein for research and therapeutic purposes. Human recombinant AHSP is used in various studies to understand its function, mechanism, and potential therapeutic applications. By studying recombinant AHSP, researchers can gain insights into its role in hemoglobin assembly and its potential use in treating hemoglobin-related disorders .

Research and Future Directions

Ongoing research on AHSP aims to further elucidate its mechanism of action and its potential therapeutic applications. Understanding how AHSP interacts with alpha-globin and other molecular partners can provide valuable insights into developing new treatments for hemoglobinopathies and other related disorders. The potential use of AHSP as a therapeutic agent in conditions like beta-thalassemia highlights the importance of continued research in this area .

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