ArticleAmerican journal of hematology2010
Analysis of alpha hemoglobin stabilizing protein overexpression in murine β-thalassemia.
Article in American journal of hematology, 2010. The graph could read no effect estimate from its abstract, so it casts no vote on the map. An erratum has been issued. Cited by 13 papers.
What it found
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
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Who cites it
13 citing papers in PubMed, 16 citations in OpenAlex.
- Association of gender and main comorbidities with expression of lncRNAs and mRNAs in COVID-19 patients.Journal, genetic engineering & biotechnology · 2026Article
- Impact of α-Globin Gene Expression and α-Globin Modifiers on the Phenotype of β-Thalassemia and Other Hemoglobinopathies: Implications for Patient Management.International journal of molecular sciences · 2024Review
- Alpha-hemoglobin-stabilizing protein (AHSP): a modulatory factor in β-thalassemia.International journal of hematology · 2020Review
- Genetic signature related to heme-hemoglobin metabolism pathway in sepsis secondary to pneumonia.NPJ systems biology and applications · 2019Article
- What can we learn from ineffective erythropoiesis in thalassemia?Blood reviews · 2018Review
- Recent challenges and advances in genetically-engineered cell therapy.Journal of pharmaceutical investigation · 2018Review
- Bidirectional immune tolerance in nonmyeloablative MHC-mismatched BMT for murine β-thalassemia.Blood · 2017Article
- Article
- Recent trends in the gene therapy of β-thalassemia.Journal of blood medicine · 2015Review
- Modulation of hepcidin as therapy for primary and secondary iron overload disorders: preclinical models and approaches.Hematology/oncology clinics of North America · 2014Review
- α-Hemoglobin-stabilizing protein (AHSP) perturbs the proximal heme pocket of oxy-α-hemoglobin and weakens the iron-oxygen bond.The Journal of biological chemistry · 2013Article
- α-Hemoglobin stabilizing protein (AHSP) markedly decreases the redox potential and reactivity of α-subunits of human HbA with hydrogen peroxide.The Journal of biological chemistry · 2013Article
- Insights into hemoglobin assembly through in vivo mutagenesis of α-hemoglobin stabilizing protein.The Journal of biological chemistry · 2012Article
Corrections and comments
- Erratum issued
Authors and funding
9 authors at 5 institutions in 1 country.
Funding
Abstract
Excess free alpha-globin is cytotoxic and contributes to the pathophysiology of b-thalassemia. Alpha hemoglobin stabilizing protein (AHSP) is a molecular chaperone that binds free alpha-globin to promote its folding and inhibit its ability to produce damaging reactive oxygen species. Reduced AHSP levels correlate with increased severity of b-thalassemia in some human cohorts, but causal mechanistic relationships are not established for these associations. We used transgenic and lentiviral gene transfer methods to investigate whether supraphysiologic AHSP levels could mitigate the severity of b-thalassemia intermedia by providing an increased sink for the excess pool of alpha-globin chains. We tested wild-type AHSP and two mutant versions with amino acid substitutions that confer 3- or 13-fold higher affinity for alpha-globin. Erythroid overexpression of these AHSP proteins up to 11-fold beyond endogenous levels had no major effects on hematologic parameters in b-thalassemic animals. Our results demonstrate that endogenous AHSP is not limiting for a-globin detoxification in a murine model of b-thalassemia.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.