Sickle‐cell haemoglobin polymerization: is it the primary pathogenic event of sickle‐cell anaemia?
PG Vekilov - British journal of haematology, 2007 - Wiley Online Library
Sickle cell anaemia is associated with a mutant haemoglobin, HbS, which forms polymers in
the red blood cells of patients. The primary role of the HbS polymerization for the …
the red blood cells of patients. The primary role of the HbS polymerization for the …
Overview of pathophysiology and rationale for treatment of sickle cell anemia.
GP Rodgers - Seminars in hematology, 1997 - europepmc.org
Sickle cell anemia occurs in individuals who are homozygous for a single nucleotide
substitution in codon 6 of the beta-globin gene. This single mutation leads to the formation of …
substitution in codon 6 of the beta-globin gene. This single mutation leads to the formation of …
Intracellular polymerization. Disease severity and therapeutic predictions.
CT Noguchi, GP Rodgers… - Annals of the New York …, 1989 - europepmc.org
The extent of intracellular polymerization of hemoglobin S, leading to loss of erythrocyte
deformability and eventual morphological sickling, is primarily determined by oxygen …
deformability and eventual morphological sickling, is primarily determined by oxygen …
Novel therapies for prevention of erythrocyte dehydration in sickle cell anemia.
C Brugnara, L De Franceschi, P Bennekou… - Drug news & …, 2001 - europepmc.org
Sickle cell anemia is a genetic disorder characterized by mutant hemoglobin (Hb)
polymerization and resultant cell deformation (sickling) under conditions of reduced oxygen …
polymerization and resultant cell deformation (sickling) under conditions of reduced oxygen …
Molecular crowding limits the role of fetal hemoglobin in therapy for sickle cell disease
M Rotter, A Aprelev, K Adachi, FA Ferrone - Journal of molecular biology, 2005 - Elsevier
The dominant assumption central to most treatments for sickle cell anemia has been that
replacement of sickle hemoglobin (HbS) by fetal hemoglobin (HbF) would have major …
replacement of sickle hemoglobin (HbS) by fetal hemoglobin (HbF) would have major …
The distinct pathobiology of sickle cell-hemoglobin C disease. Therapeutic implications.
RL Nagel, C Lawrence - Hematology/oncology clinics of North …, 1991 - europepmc.org
The data available clearly establish that the hyperconcentration of hemoglobin C and S
inside SC cells is the main and driving mechanism for the pathologic behavior of these cells …
inside SC cells is the main and driving mechanism for the pathologic behavior of these cells …
Polymerization and sickle cell disease: a molecular view
FA Ferrone - Microcirculation, 2004 - Taylor & Francis
The present molecular-level understanding of polymerization and sickling is reviewed for 2
central questions in sickle hemoglobin pathophysiology, viz., what determines when cells …
central questions in sickle hemoglobin pathophysiology, viz., what determines when cells …
Pathophysiology of sickle cell disease: role of cellular and genetic modifiers
MH Steinberg, GP Rodgers - Seminars in hematology, 2001 - Elsevier
Sickle hemoglobin (HbS), caused by a point mutation in the β-globin gene of hemoglobin,
polymerizes when deoxygenated. The pathophysiology of sickle cell disease results from …
polymerizes when deoxygenated. The pathophysiology of sickle cell disease results from …
Molecular and cellular pathophysiology of sickle cell anemia
D Labie, J Elion - Pathologie-biologie, 1999 - europepmc.org
The beta S mutation responsible for sickle cell disease (SCD) was identified in 1949. This
mutation always consists in a T for A substitution at codon 6 of the beta-globin chain …
mutation always consists in a T for A substitution at codon 6 of the beta-globin chain …