Property Summary

NCBI Gene PubMed Count 41
PubMed Score 96.49
PubTator Score 595.25

Knowledge Summary

Patent

No data available

TINX Plot

Expression

  Differential Expression (5)

Disease log2 FC p
osteosarcoma -5.898 2.7e-06
tuberculosis 1.100 4.4e-02
mucosa-associated lymphoid tissue lympho... 3.884 9.3e-03
acute myeloid leukemia -1.500 2.3e-02
psoriasis -2.500 2.9e-17

Gene RIF (33)

PMID Text
26460260 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.
25648458 In maturing RBC progenitors AHSP bind to free alpha-globin chains to increase the HbA production. (Review)
25611244 AHSP is predominantly expressed in erythroid precursors in bone marrow biopsy specimens from patients with hematologic malignancies.
24795058 The relationship between AHSP gene expression, disease severity, and the beta/alpha globin mRNA ratio was studied among different homozygote beta-thalassemia patients.
24740453 analysis showed binding of STAT3 to AHSP promoter and binding was significantly augmented with IL6 stimulation and upon alpha-globin overexpression
23696640 alpha-Hemoglobin-stabilizing protein (AHSP) perturbs the proximal heme pocket of oxy-alpha-hemoglobin and weakens the iron-oxygen bond.
23333304 HIV-1 Vif upregulates the expression of alpha hemoglobin stabilizing protein (AHSP) in Vif-expression T cells
23264625 alpha-Hemoglobin stabilizing protein (AHSP) markedly decreases the redox potential and reactivity of alpha-subunits of human HbA with hydrogen peroxide.
22298770 AHSP acts as a molecular chaperone by rapidly binding and stabilizing met-alpha hemichrome folding intermediates
22079025 AHSP could be a secondary compensatory mechanism in red blood cells to counterbalance the excess of alpha-globin chains in HbE/beta-thalassaemia individuals.
21232177 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
20815047 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.
20627634 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.
20627634 Observational study of gene-disease association. (HuGE Navigator)
20371604 analysis of the action of a human mutant, AHSPV56G, of alpha-hemoglobin stabilizing protein (AHSP)
20036801 Studies indicate that the interaction of alpha-Hb with AHSP involves surfaces normally employed in binding to beta-Hb.
19706593 A cis-proline in alpha-hemoglobin stabilizing protein directs the structural reorganization of alpha-hemoglobin.
19482015 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.
19349619 AHSP promotes alpha globin chain stability during human erythropoiesis
18704762 Reduced AHSP may identify women at risk of experiencing further miscarriages.
18676996 An iron responsive element-like stem-loop regulates alpha-hemoglobin-stabilizing protein mRNA.
18347943 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.
18179859 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.
18166800 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
17874450 The 12391 G>A SNP is common and represents a potential mechanism through which genetically determined variations in AHSP expression could influence beta-thalassemia.
17194704 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
16339656 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.
16186125 identified an AHSP gene erythroid promoter with functionally important binding sites for GATA-1- and Oct-1-related proteins
15220346 AHSP is a chaperone for transfer of human alpha- to beta-hemoglobin
14715623 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
14667971 progesterone, corticotropin-releasing factor, and activin A have roles in paracrine regulation of endometrial function [review]
12192002 determination as a predominantly alpha-helical globular protein with a somewhat asymmetric shape
12066189 An abundant erythroid protein that stabilizes free alpha-haemoglobin.

AA Sequence

MALLKANKDLISAGLKEFSVLLNQQVFNDPLVSEEDMVTVVEDWMNFYINYYRQQVTGEPQERDKALQEL      1 - 70
RQELNTLANPFLAKYRDFLKSHELPSHPPPSS                                           71 - 102
//

Text Mined References (43)

PMID Year Title
26460260 2015 Study of alpha hemoglobin stabilizing protein expression in patients with ? thalassemia and sickle cell anemia and its impact on clinical severity.
25648458 2015 Review: Beta-thalassemia and molecular chaperones.
25611244 2016 ?-Hemoglobin-stabilizing Protein: An Effective Marker for Erythroid Precursors in Bone Marrow Biopsy Specimens.
25416956 2014 A proteome-scale map of the human interactome network.
24795058 2014 Relationship between AHSP gene expression, ?/? globin mRNA ratio, and clinical severity of the ?-thalassemia patients.
24740453 2014 Activation of STAT3 stimulates AHSP expression in K562 cells.
23696640 2013 ?-Hemoglobin-stabilizing protein (AHSP) perturbs the proximal heme pocket of oxy-?-hemoglobin and weakens the iron-oxygen bond.
23264625 2013 ?-Hemoglobin stabilizing protein (AHSP) markedly decreases the redox potential and reactivity of ?-subunits of human HbA with hydrogen peroxide.
22298770 2012 Kinetics of ?-globin binding to ?-hemoglobin stabilizing protein (AHSP) indicate preferential stabilization of hemichrome folding intermediate.
22079025 2012 ?-Haemoglobin stabilising protein expression is influenced by mean cell haemoglobin and HbF levels in HbE/?-thalassaemia individuals.
21232177 2010 NF-E2: a novel regulator of alpha-hemoglobin stabilizing protein gene expression.
20815047 2010 Analysis of alpha hemoglobin stabilizing protein overexpression in murine ?-thalassemia.
20627634 2010 Analysis of alpha-hemoglobin-stabilizing protein (AHSP) gene as a genetic modifier to the phenotype of beta-thalassemia in Southern China.
20371604 2010 Alpha-hemoglobin stabilizing protein (AHSP), a kinetic scheme of the action of a human mutant, AHSPV56G.
20036801 2010 Alpha-hemoglobin stabilizing protein: molecular function and clinical correlation.
19706593 2009 A cis-proline in alpha-hemoglobin stabilizing protein directs the structural reorganization of alpha-hemoglobin.
19482015 2009 The alpha-hemoglobin stabilizing protein and expression of unstable alpha-Hb variants.
19349619 2009 Analysis of human alpha globin gene mutations that impair binding to the alpha hemoglobin stabilizing protein.
18704762 2009 Placental Alpha Hemoglobin Stabilizing Protein (AHSP) and recurrent miscarriage.
18676996 2008 An iron responsive element-like stem-loop regulates alpha-hemoglobin-stabilizing protein mRNA.
18347943 2008 Alpha-hemoglobin-stabilizing protein (AHSP) in hemolysis, elevated liver enzyme, and low platelet (HELLP) syndrome, intrauterine growth restriction (IUGR) and fetal death.
18179859 2008 Reduction of AHSP synthesis in hemin-induced K562 cells and EPO-induced CD34(+) cells leads to alpha-globin precipitation, impairment of normal hemoglobin production, and increased cell death.
18166800 2008 Hb Foggia or alpha 117(GH5)Phe -> Ser: a new alpha 2 globin allele affecting the alpha Hb-AHSP interaction.
17874450 2008 Population analysis of the alpha hemoglobin stabilizing protein (AHSP) gene identifies sequence variants that alter expression and function.
17194704 2007 Reversible hexacoordination of alpha-hemoglobin-stabilizing protein (AHSP)/alpha-hemoglobin Versus pressure. Evidence for protection of the alpha-chains by their chaperone.
17192395 2007 Comparative gene expression profiling of in vitro differentiated megakaryocytes and erythroblasts identifies novel activatory and inhibitory platelet membrane proteins.
16339656 2005 Role of alpha-hemoglobin-stabilizing protein in normal erythropoiesis and beta-thalassemia.
16189514 2005 Towards a proteome-scale map of the human protein-protein interaction network.
16186125 2005 GATA-1 and Oct-1 are required for expression of the human alpha-hemoglobin-stabilizing protein gene.
15931225 2005 Structure of oxidized alpha-haemoglobin bound to AHSP reveals a protective mechanism for haem.
15550245 2004 Molecular mechanism of AHSP-mediated stabilization of alpha-hemoglobin.
15489334 2004 The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC).
15220346 2004 Transfer of human alpha- to beta-hemoglobin via its chaperone protein: evidence for a new state.
15178680 2004 NMR structure of the alpha-hemoglobin stabilizing protein: insights into conformational heterogeneity and binding.
14715623 2004 Evaluation of alpha hemoglobin stabilizing protein (AHSP) as a genetic modifier in patients with beta thalassemia.
14667971 2003 Paracrine regulation of endometrial function: interaction between progesterone and corticotropin-releasing factor (CRF) and activin A.
12477932 2002 Generation and initial analysis of more than 15,000 full-length human and mouse cDNA sequences.
12192002 2002 Biophysical characterization of the alpha-globin binding protein alpha-hemoglobin stabilizing protein.
12066189 2002 An abundant erythroid protein that stabilizes free alpha-haemoglobin.
11231637 2001 A novel erythroid-specific marker of transmissible spongiform encephalopathies.
11042152 2000 Cloning and functional analysis of cDNAs with open reading frames for 300 previously undefined genes expressed in CD34+ hematopoietic stem/progenitor cells.
10409428 1999 Gene expression in proliferating human erythroid cells.
8535075 1995 Construction of a gene expression profile of a human fetal liver by single-pass cDNA sequencing.