The alpha-hemoglobin stabilizing protein and expression of unstable alpha-Hb variants.

@article{Vasseur2009TheAS,
  title={The alpha-hemoglobin stabilizing protein and expression of unstable alpha-Hb variants.},
  author={C. Vasseur and {\'E}lisa Domingues-Hamdi and Thomas Brillet and M. Marden and V. Baudin-Creuza},
  journal={Clinical biochemistry},
  year={2009},
  volume={42 18},
  pages={
          1818-23
        }
}
  • C. Vasseur, Élisa Domingues-Hamdi, +2 authors V. Baudin-Creuza
  • Published 2009
  • Chemistry, Medicine
  • Clinical biochemistry
    • OBJECTIVES To determine the role of the alpha-hemoglobin stabilizing protein (AHSP) in the clinical expression of alpha-hemoglobin (alpha-Hb) variants described as unstable, ten alpha chain variants have been studied with their chaperone. AHSP specifically binds free alpha-Hb to form a soluble heterodimer until it is replaced by the beta-Hb partner. In this way, AHSP prevents the precipitation of free alpha chains which might damage the membrane of erythrocyte. AHSP specifically recognizes the… CONTINUE READING
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    20 Citations
    Background Citations
    7
    Methods Citations
    1
    Results Citations
    1
    20 Citations
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    Citation Type

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    References

    SHOWING 1-10 OF 38 REFERENCES
    Impaired binding of AHSP to α chain variants: Hb Groene Hart illustrates a mechanism leading to unstable hemoglobins with α thalassemic like syndrome
    • 35
    Molecular Mechanism of AHSP-Mediated Stabilization of α-Hemoglobin
    • 114
    An erythroid chaperone that facilitates folding of α-globin subunits for hemoglobin synthesis
    • 88
    • PDF
    Biophysical Characterization of the α-Globin Binding Protein α-Hemoglobin Stabilizing Protein*
    • 91
    • PDF
    Transfer of Human α- to β-Hemoglobin via Its Chaperone Protein
    • 29
    • PDF
    High-yield expression in Escherichia coli of soluble human alpha-hemoglobin complexed with its molecular chaperone.
    • 37
    • PDF
    Loss of α-hemoglobin–stabilizing protein impairs erythropoiesis and exacerbates β-thalassemia
    • 124
    • PDF
    Expression of α-hemoglobin stabilizing protein gene during human erythropoiesis
    • 40
    Population analysis of the alpha hemoglobin stabilizing protein (AHSP) gene identifies sequence variants that alter expression and function
    • 40
    Molecular basis for alpha-thalassemia associated with the structural mutant hemoglobin Suan-Dok (alpha 2 109leu----arg)
    • 20
    • PDF