Sickle cell disease: the proportion of liganded haemoglobin needed to prevent crises

IM Franklin, MA Rosemeyer… - British Journal of …, 1983 - Wiley Online Library
In an attempt to predict the likelihood of successfully treating sickle cell disease by
increasing haemoglobin S (Hb S) oxygen affinity, two liganded derivatives of Hb S have …

Pharmacological modification of oxygen affinity improves deformability of deoxygenated sickle erythrocytes: a possible therapeutic approach to sickle cell disease.

AJ Keidan, MC Sowter, CS Johnson… - Clinical science …, 1989 - europepmc.org
1. The formation of polymerized haemoglobin S in sickle cells is critically dependent on the
concentration of deoxygenated haemoglobin so that compounds which increase the oxygen …

Intracellular polymerization of sickle hemoglobin: disease severity and therapeutic goals.

CT Noguchi, GP Rodgers… - Progress in Clinical and …, 1987 - europepmc.org
We have demonstrated that the extent of intracellular polymerization of deoxyhemoglobin S
can be predicted from knowledge of intracellular hemoglobin concentration, composition …

The effect of urea on sickling

A May, ER Huehns - British Journal of Haematology, 1975 - Wiley Online Library
The effect of urea on the oxygen affinity of sickle cells and normal cells was studied up to a
concentration of about 1.0 M. Besides the increase in oxygen affinity found in both normal …

[引用][C] Treatment of sickle-cell disease

A May, ER Huehns - Transactions of the Royal Society of …, 1974 - academic.oup.com
Sickle-cell disease arises from the homozygous inheritance of genes which synthesize Hb-S
rather than Hb-A. Its prevention and treatment are of great importance both because of its …

The determinants of irreversibly sickled cells in homozygous sickle cell disease

GR Serjeant, E Serjeant, P Desai… - British Journal of …, 1978 - Wiley Online Library
The relationship between the irreversibly sickled cell (ISC) count and other haematological
parameters has been investigated. Positive correlations occurred with the MCH, MCHC, and …

[引用][C] Treatment of sickle‐cell disease

RG Huntsman, H Lehmann - British journal of haematology, 1974 - Wiley Online Library
The term sickle-cell disease includes any state where the proportion of haemoglobin S is
greater than 50%(eg sickle-cell anaemia, sickle-cell haemoglobin C disease and sickle-cell …

Intracellular polymerization of sickle hemoglobin. Effects of cell heterogeneity.

CT Noguchi, DA Torchia… - The Journal of clinical …, 1983 - Am Soc Clin Investig
To determine the extent to which the broad distribution in intracellular hemoglobin
concentrations found in sickle erythrocytes affects the extent of intracellular polymerization of …

Determination of deoxyhemoglobin S polymer in sickle erythrocytes upon deoxygenation.

CT Noguchi, DA Torchia… - Proceedings of the …, 1980 - National Acad Sciences
We have used 13C/1H magnetic double-resonance spectroscopy to measure the amount of
sickle hemoglobin polymer within sickle erythrocytes as a function of oxygen saturation. We …

Molecular and cellular pathogenesis of hemoglobin SC disease.

HF Bunn, CT Noguchi, J Hofrichter… - Proceedings of the …, 1982 - National Acad Sciences
Solution and cell studies were performed to ascertain why individuals with hemoglobin (Hb)
SC have disease whereas those with Hb AS do not. The polymerization of deoxygenated …