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  4. Recombinant Human Alpha-hemoglobin-stabilizing protein (AHSP)

Recombinant Human Alpha-hemoglobin-stabilizing protein (AHSP)

  • 中文名称：
    Recombinant Human Alpha-hemoglobin-stabilizing protein(AHSP)
  • 货号：
    CSB-EP878935HU
  • 规格：
    ￥1344
  • 图片：
    • (Tris-Glycine gel) Discontinuous SDS-PAGE (reduced) with 5% enrichment gel and 15% separation gel.
  • 其他：

产品详情

  • 纯度：
    Greater than 90% as determined by SDS-PAGE.
  • 基因名：
    AHSP
  • Uniprot No.：
  • 别名：
    AHSP; AHSP_HUMAN; Alpha hemoglobin stabilizing protein; Alpha-hemoglobin-stabilizing protein; EDRF; ERAF; Erythroid associated factor; Erythroid differentiation associated factor; Erythroid differentiation related factor; Erythroid differentiation-related factor; Erythroid-associated factor
  • 种属：
    Homo sapiens (Human)
  • 蛋白长度：
    Full Length
  • 来源：
    E.coli
  • 分子量：
    38.8kDa
  • 表达区域：
    1-102aa
  • 氨基酸序列
    MALLKANKDLISAGLKEFSVLLNQQVFNDPLVSEEDMVTVVEDWMNFYINYYRQQVTGEPQERDKALQELRQELNTLANPFLAKYRDFLKSHELPSHPPPSS
    Note: The complete sequence including tag sequence, target protein sequence and linker sequence could be provided upon request.
  • 蛋白标签：
    N-terminal GST-tagged
  • 产品提供形式：
    Liquid or Lyophilized powder
    Note: We will preferentially ship the format that we have in stock, however, if you have any special requirement for the format, please remark your requirement when placing the order, we will prepare according to your demand.
  • 缓冲液：
    Tris-based buffer,50% glycerol
  • 储存条件：
    Store at -20°C/-80°C upon receipt, aliquoting is necessary for mutiple use. Avoid repeated freeze-thaw cycles.
  • 保质期：
    The shelf life is related to many factors, storage state, buffer ingredients, storage temperature and the stability of the protein itself.
    Generally, the shelf life of liquid form is 6 months at -20°C/-80°C. The shelf life of lyophilized form is 12 months at -20°C/-80°C.
  • 货期：
    Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time may differ from different purchasing way or location, please kindly consult your local distributors for specific delivery time.
    Note: All of our proteins are default shipped with normal blue ice packs, if you request to ship with dry ice, please communicate with us in advance and extra fees will be charged.
  • 注意事项：
    Repeated freezing and thawing is not recommended. Store working aliquots at 4°C for up to one week.
  • Datasheet & COA：
    Please contact us to get it.

产品评价

靶点详情

  • 功能：
    Acts as a chaperone to prevent the harmful aggregation of alpha-hemoglobin during normal erythroid cell development. Specifically protects free alpha-hemoglobin from precipitation. It is predicted to modulate pathological states of alpha-hemoglobin excess such as beta-thalassemia.
  • 基因功能参考文献：
    1. In the presence of free alpha subunits and H2O2, both HbA and HbE showed bCys93 oxidation which increased with higher H2O2 concentrations. In the presence of Alpha-hemoglobin stabilizing protein (AHSP)Cys93 oxidation was substantially reduced in both proteins.in the presence of excess free alpha-subunit and under the same oxidative conditions, these events are substantially increased for HbE compared to HbA PMID: 26995402
    2. Findings indicate that alpha-hemoglobin-stabilizing protein (AHSP) expression is a biomarker of hemoglobin H (HbH) disease severity and infer an important role of AHSP in modulating the pathophysiology of this disease. PMID: 28337528
    3. AHSP is predominantly expressed in erythroid precursors in bone marrow biopsy specimens from patients with hematologic malignancies. PMID: 25611244
    4. AHSP expression was higher in patients with sickle cell anemia versus thalassemia, with no significant difference between BTM and BTI. Expression was higher in patients with NTDT and on hydroxyurea therapy. PMID: 26460260
    5. In maturing RBC progenitors AHSP bind to free alpha-globin chains to increase the HbA production. (Review) PMID: 25648458
    6. analysis showed binding of STAT3 to AHSP promoter and binding was significantly augmented with IL6 stimulation and upon alpha-globin overexpression PMID: 24740453
    7. The relationship between AHSP gene expression, disease severity, and the beta/alpha globin mRNA ratio was studied among different homozygote beta-thalassemia patients. PMID: 24795058
    8. alpha-Hemoglobin-stabilizing protein (AHSP) perturbs the proximal heme pocket of oxy-alpha-hemoglobin and weakens the iron-oxygen bond. PMID: 23696640
    9. alpha-Hemoglobin stabilizing protein (AHSP) markedly decreases the redox potential and reactivity of alpha-subunits of human HbA with hydrogen peroxide. PMID: 23264625
    10. AHSP acts as a molecular chaperone by rapidly binding and stabilizing met-alpha hemichrome folding intermediates PMID: 22298770
    11. AHSP could be a secondary compensatory mechanism in red blood cells to counterbalance the excess of alpha-globin chains in HbE/beta-thalassaemia individuals. PMID: 22079025
    12. NF-E2 may play an important role in AHSP gene regulation, providing new insights into the molecular mechanisms underlying the erythroid-specific expression of AHSP as well as new possibilities for beta-thalassemia treatment PMID: 21232177
    13. No significant association has been found between specific AHSP alleles or haplotypes and the disease severity of beta-thalassemia. Our study suggested that AHSP is not a significant genetic modifier of beta-thalassemia in southern China. PMID: 20627634
    14. Overexpression of human AHSP & 2 mutant versions with AA substitutions confering 3- or 13-fold higher affinity for alpha-globin had no major effects on hematologic parameters in beta-thalassemic mice. PMID: 20815047
    15. analysis of the action of a human mutant, AHSPV56G, of alpha-hemoglobin stabilizing protein (AHSP) PMID: 20371604
    16. Studies indicate that the interaction of alpha-Hb with AHSP involves surfaces normally employed in binding to beta-Hb. PMID: 20036801
    17. Different mechanisms may be responsible for the amount of abnormal Hb recovered, such as a highly unstable alpha chain or an impaired formation of the complex AHSP/alpha-Hb or a modification of the alphabeta dimer formation. PMID: 19482015
    18. An abundant erythroid protein that stabilizes free alpha-haemoglobin. PMID: 12066189
    19. determination as a predominantly alpha-helical globular protein with a somewhat asymmetric shape PMID: 12192002
    20. progesterone, corticotropin-releasing factor, and activin A have roles in paracrine regulation of endometrial function [review] PMID: 14667971
    21. Using gene mapping, direct genomic sequencing, and extended haplotype analysis, no mutation or specific association between haplotypes of AHSP and disease severity was found, suggesting that AHSP is not a disease modifier in Hb E-beta thalassemia PMID: 14715623
    22. AHSP is a chaperone for transfer of human alpha- to beta-hemoglobin PMID: 15220346
    23. identified an AHSP gene erythroid promoter with functionally important binding sites for GATA-1- and Oct-1-related proteins PMID: 16186125
    24. Review. AHSP specifically binds free alphaHb, stabilizes its structure, & limits its ability to generate reactive oxygen species. It binds the G & H helices of alphaHb on a surface that largely overlaps with the alpha1-beta1 interface of HbA. PMID: 16339656
    25. results reveal a plasticity of the alpha-Hb active site in the presence of the chaperone AHSP and indicate that the AHSP was still active at 300 MPa PMID: 17194704
    26. The 12391 G>A SNP is common and represents a potential mechanism through which genetically determined variations in AHSP expression could influence beta-thalassemia. PMID: 17874450
    27. the alpha2-globin mutation cod 117 TTC>TCC or alpha 117(GH5)Phe>Ser impairs the interaction of the alpha-chain variant with the AHSP and prevents its stabilizing effect, thus leading to the alpha-chain pool reduction PMID: 18166800
    28. The AHSP stabilizes the alphaHb chain, avoiding its precipitation and its ability to generate ROS, which implicate in cell death.Data indicate that AHSP may be significant for human hemoglobin formation and it is a key protein during human erythropoiesis. PMID: 18179859
    29. Placental AHSP mRNA level in HELLP & intrauterine fetal death were significantly decreased compared with controls. It may be involved in the pathogenic mechanisms leading to the adverse pregnancy outcome. PMID: 18347943
    30. An iron responsive element-like stem-loop regulates alpha-hemoglobin-stabilizing protein mRNA. PMID: 18676996
    31. Reduced AHSP may identify women at risk of experiencing further miscarriages. PMID: 18704762
    32. AHSP promotes alpha globin chain stability during human erythropoiesis PMID: 19349619
    33. A cis-proline in alpha-hemoglobin stabilizing protein directs the structural reorganization of alpha-hemoglobin. PMID: 19706593

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  • 亚细胞定位：
    Cytoplasm.
  • 蛋白家族：
    AHSP family
  • 组织特异性：
    Expressed in blood and bone marrow.
  • 数据库链接：

    HGNC: 18075

    OMIM: 605821

    KEGG: hsa:51327

    STRING: 9606.ENSP00000307199

    UniGene: Hs.274309