| Alpha haemoglobin-stabilising protein concentration in the red blood cells of patients with sickle cell anaemia with and without hydroxycarbamide treatment. | Alpha haemoglobin-stabilising protein concentration in the red blood cells of patients with sickle cell anaemia with and without hydroxycarbamide treatment.
Vasseur C, Domingues-Hamdi E, Pakdaman S, Galactéros F, Baudin-Creuza V. | 02/19/2022 |
| The study documents that among the HbE beta thalassemia patients with varying severity, an exon mutation in AHSP is significantly prevalent only among the transfusion-dependent beta-thalassemia patients. | Association of alpha hemoglobin-stabilizing protein (AHSP) gene mutation and disease severity among HbE-beta thalassemia patients.
Ray R, Kalantri SA, Bhattacharjee S, Biswas A, Shahab A, Biswas S, Bhattacharyya M. | 07/27/2019 |
| 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 | Oxidative instability of hemoglobin E (β26 Glu→Lys) is increased in the presence of free α subunits and reversed by α-hemoglobin stabilizing protein (AHSP): Relevance to HbE/β-thalassemia.
Strader MB, Kassa T, Meng F, Wood FB, Hirsch RE, Friedman JM, Alayash AI., Free PMC Article | 11/25/2017 |
| 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. | A molecular study on the role of alpha-hemoglobin-stabilizing protein in hemoglobin H disease.
Surapolchai P, Chuansumrit A, Sirachainan N, Kadegasem P, Leung KC, So CC. | 08/19/2017 |
| AHSP is predominantly expressed in erythroid precursors in bone marrow biopsy specimens from patients with hematologic malignancies. | α-Hemoglobin-stabilizing Protein: An Effective Marker for Erythroid Precursors in Bone Marrow Biopsy Specimens.
Yu H, Pinkus JL, Pinkus GS. | 09/17/2016 |
| 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. | Study of alpha hemoglobin stabilizing protein expression in patients with β thalassemia and sickle cell anemia and its impact on clinical severity.
Mahmoud HM, Shoeib AA, Abd El Ghany SM, Reda MM, Ragab IA. | 07/30/2016 |
| In maturing RBC progenitors AHSP bind to free alpha-globin chains to increase the HbA production. (Review) | Review: Beta-thalassemia and molecular chaperones.
Sumera A, Radhakrishnan A, Baba AA, George E. | 12/26/2015 |
| analysis showed binding of STAT3 to AHSP promoter and binding was significantly augmented with IL6 stimulation and upon alpha-globin overexpression | Activation of STAT3 stimulates AHSP expression in K562 cells.
Cao C, Zhao G, Yu W, Xie X, Wang W, Yang R, Lv X, Liu D. | 05/16/2015 |
| The relationship between AHSP gene expression, disease severity, and the beta/alpha globin mRNA ratio was studied among different homozygote beta-thalassemia patients. | Relationship between AHSP gene expression, β/α globin mRNA ratio, and clinical severity of the β-thalassemia patients.
Ranjbaran R, Okhovat MA, Mobarhanfard A, Aboualizadeh F, Abbasi M, Moezzi L, Golafshan HA, Behzad-Behbahani A, Bagheri M, Sharifzadeh S. | 12/20/2014 |
| alpha-Hemoglobin-stabilizing protein (AHSP) perturbs the proximal heme pocket of oxy-alpha-hemoglobin and weakens the iron-oxygen bond. | α-Hemoglobin-stabilizing protein (AHSP) perturbs the proximal heme pocket of oxy-α-hemoglobin and weakens the iron-oxygen bond.
Dickson CF, Rich AM, D'Avigdor WM, Collins DA, Lowry JA, Mollan TL, Khandros E, Olson JS, Weiss MJ, Mackay JP, Lay PA, Gell DA., Free PMC Article | 09/14/2013 |
| alpha-Hemoglobin stabilizing protein (AHSP) markedly decreases the redox potential and reactivity of alpha-subunits of human HbA with hydrogen peroxide. | α-Hemoglobin stabilizing protein (AHSP) markedly decreases the redox potential and reactivity of α-subunits of human HbA with hydrogen peroxide.
Mollan TL, Banerjee S, Wu G, Parker Siburt CJ, Tsai AL, Olson JS, Weiss MJ, Crumbliss AL, Alayash AI., Free PMC Article | 04/20/2013 |
| AHSP acts as a molecular chaperone by rapidly binding and stabilizing met-alpha hemichrome folding intermediates | Kinetics of α-globin binding to α-hemoglobin stabilizing protein (AHSP) indicate preferential stabilization of hemichrome folding intermediate.
Mollan TL, Khandros E, Weiss MJ, Olson JS., Free PMC Article | 09/22/2012 |
| AHSP could be a secondary compensatory mechanism in red blood cells to counterbalance the excess of alpha-globin chains in HbE/beta-thalassaemia individuals. | α-Haemoglobin stabilising protein expression is influenced by mean cell haemoglobin and HbF levels in HbE/β-thalassaemia individuals.
Lim WF, Muniandi L, George E, Sathar J, Teh LK, Gan GG, Lai MI. | 09/15/2012 |
| 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 | NF-E2: a novel regulator of alpha-hemoglobin stabilizing protein gene expression.
Guo-wei Z, Rui-feng Y, Xiang L, Mitchell WJ, De-pei L, Chih-chuan L. | 04/16/2011 |
| 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. | Analysis of alpha-hemoglobin-stabilizing protein (AHSP) gene as a genetic modifier to the phenotype of beta-thalassemia in Southern China.
Wang Z, Yu W, Li Y, Shang X, Zhang X, Xiong F, Xu X, Wang Z, Yu W, Li Y, Shang X, Zhang X, Xiong F, Xu X. | 12/11/2010 |
| 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. | Analysis of alpha hemoglobin stabilizing protein overexpression in murine β-thalassemia.
Nasimuzzaman M, Khandros E, Wang X, Kong Y, Zhao H, Weiss D, Rivella S, Weiss MJ, Persons DA., Free PMC Article | 10/23/2010 |
| Observational study of gene-disease association. (HuGE Navigator) | Analysis of alpha-hemoglobin-stabilizing protein (AHSP) gene as a genetic modifier to the phenotype of beta-thalassemia in Southern China.
Wang Z, Yu W, Li Y, Shang X, Zhang X, Xiong F, Xu X, Wang Z, Yu W, Li Y, Shang X, Zhang X, Xiong F, Xu X. | 09/15/2010 |
| analysis of the action of a human mutant, AHSPV56G, of alpha-hemoglobin stabilizing protein (AHSP) | Alpha-hemoglobin stabilizing protein (AHSP), a kinetic scheme of the action of a human mutant, AHSPV56G.
Brillet T, Baudin-Creuza V, Vasseur C, Domingues-Hamdi E, Kiger L, Wajcman H, Pissard S, Marden MC., Free PMC Article | 06/28/2010 |
| Studies indicate that the interaction of alpha-Hb with AHSP involves surfaces normally employed in binding to beta-Hb. | Alpha-hemoglobin stabilizing protein: molecular function and clinical correlation.
Turbpaiboon C, Wilairat P. | 05/3/2010 |
| 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. | The alpha-hemoglobin stabilizing protein and expression of unstable alpha-Hb variants.
Vasseur C, Domingues-Hamdi E, Brillet T, Marden MC, Baudin-Creuza V. | 02/8/2010 |
| A cis-proline in alpha-hemoglobin stabilizing protein directs the structural reorganization of alpha-hemoglobin. | A cis-proline in alpha-hemoglobin stabilizing protein directs the structural reorganization of alpha-hemoglobin.
Gell DA, Feng L, Zhou S, Jeffrey PD, Bendak K, Gow A, Weiss MJ, Shi Y, Mackay JP., Free PMC Article | 01/21/2010 |
| 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. | Alpha-hemoglobin-stabilizing protein (AHSP) in hemolysis, elevated liver enzyme, and low platelet (HELLP) syndrome, intrauterine growth restriction (IUGR) and fetal death.
Emanuelli M, Sartini D, Rossi V, Corradetti A, Landi B, Vianna CR, Giannubilo S, Tranquilli AL., Free PMC Article | 01/21/2010 |
| AHSP promotes alpha globin chain stability during human erythropoiesis | Analysis of human alpha globin gene mutations that impair binding to the alpha hemoglobin stabilizing protein.
Yu X, Mollan TL, Butler A, Gow AJ, Olson JS, Weiss MJ., Free PMC Article | 01/21/2010 |
| Reduced AHSP may identify women at risk of experiencing further miscarriages. | Placental Alpha Hemoglobin Stabilizing Protein (AHSP) and recurrent miscarriage.
Emanuelli M, Cecati M, Sartini D, Stortoni P, Corradetti A, Giannubilo SR, Turi A, Tranquilli AL., Free PMC Article | 01/21/2010 |
| An iron responsive element-like stem-loop regulates alpha-hemoglobin-stabilizing protein mRNA. | An iron responsive element-like stem-loop regulates alpha-hemoglobin-stabilizing protein mRNA.
dos Santos CO, Dore LC, Valentine E, Shelat SG, Hardison RC, Ghosh M, Wang W, Eisenstein RS, Costa FF, Weiss MJ., Free PMC Article | 01/21/2010 |