A Role for the α113 (GH1) Amino Acid Residue in the Polymerization of Sickle Hemoglobin: EVALUATION OF ITS INHIBITORY STRENGTH AND INTERACTION …
MVS Sivaram, R Sudha, RP Roy - Journal of Biological Chemistry, 2001 - ASBMB
A cluster of amino acid residues located in the AB-GH region of the α-chain are shown in
intra-double strand axial interactions of the hemoglobin S (HbS) polymer. However, αLeu …
intra-double strand axial interactions of the hemoglobin S (HbS) polymer. However, αLeu …
Band 3 catalyzes sickle hemoglobin polymerization
We have measured homogeneous and heterogeneous nucleation rates of sickle
hemoglobin (HbS) in the presence of a strongly binding deletion mutant of the cytoplasmic …
hemoglobin (HbS) in the presence of a strongly binding deletion mutant of the cytoplasmic …
Minireview: Genetic basis of heterogeneity and severity in sickle cell disease
A Habara, MH Steinberg - Experimental Biology and …, 2016 - journals.sagepub.com
Sickle cell disease, a common single gene disorder, has a complex pathophysiology that at
its root is initiated by the polymerization of deoxy sickle hemoglobin. Sickle vasoocclusion …
its root is initiated by the polymerization of deoxy sickle hemoglobin. Sickle vasoocclusion …
Reversing the hemoglobin switch
VG Sankaran, DG Nathan - New England Journal of Medicine, 2010 - Mass Medical Soc
Natural observations can often provide important clues for how therapies can be developed
to ameliorate disease. Disorders of β-hemoglobin, including sickle cell disease and β …
to ameliorate disease. Disorders of β-hemoglobin, including sickle cell disease and β …
Understanding heterogeneity of fetal hemoglobin induction through comparative analysis of F and A erythroblasts
Reversing the developmental switch from fetal hemoglobin (HbF, α2γ2) to adult hemoglobin
(HbA, α2β2) is an important therapeutic approach in sickle cell disease (SCD) and β …
(HbA, α2β2) is an important therapeutic approach in sickle cell disease (SCD) and β …
Evidence for carbon monoxide binding to sickle cell polymers during melting
SK Aroutiounian, JG Louderback, SK Ballas… - Biophysical …, 2001 - Elsevier
The melting of sickle cell hemoglobin (HbS) polymers was induced by rapid dilution using a
stopped-flow apparatus. The kinetics of polymer melting were monitored using light …
stopped-flow apparatus. The kinetics of polymer melting were monitored using light …
High-throughput assessment of hemoglobin polymer in single red blood cells from sickle cell patients under controlled oxygen tension
G Di Caprio, E Schonbrun… - Proceedings of the …, 2019 - National Acad Sciences
Sickle cell disease (SCD) is caused by a variant hemoglobin molecule that polymerizes
inside red blood cells (RBCs) in reduced oxygen tension. Treatment development has been …
inside red blood cells (RBCs) in reduced oxygen tension. Treatment development has been …
Fetal hemoglobin in sickle hemoglobinopathies: high HbF genotypes and phenotypes
MH Steinberg - Journal of Clinical Medicine, 2020 - mdpi.com
Fetal hemoglobin (HbF) usually consists of 4 to 10% of total hemoglobin in adults of African
descent with sickle cell anemia. Rarely, their HbF levels reach more than 30%. High HbF …
descent with sickle cell anemia. Rarely, their HbF levels reach more than 30%. High HbF …
Gelation of sickle cell hemoglobin in mixtures with normal adult and fetal hemoglobins
HR Sunshine, J Hofrichter, WA Eaton - Journal of molecular biology, 1979 - Elsevier
We report the results of thermodynamic and kinetic studies on the gelation of mixtures of
sickle cell (S) deoxyhemoglobin with normal human adult (A) and fetal (F) …
sickle cell (S) deoxyhemoglobin with normal human adult (A) and fetal (F) …