Results 201 to 210 of about 46,454 (250)

A Novel Sickle Hemoglobin: Hemoglobin S-South End

Journal of Pediatric Hematology/Oncology, 2004
Sickle hemoglobin (Hb S; beta Glu6Val) is due to an AGTG; beta Lys132Asn, AAA>AAC). When present alone, the beta Lys132Asn mutation has low oxygen affinity. Therefore, this mutation may enhance the polymerization of the Hb S variant. Furthermore, the variant hemoglobin mimics Hb A on high-pressure liquid chromatography, and its identity is not easily ...
Hong-Yuan, Luo, Adeboye H, Adewoye, Shawn H, Eung, Timothy P, Skelton, Karen, Quillen, Lillian, McMahon, Martin H, Steinberg, David H K, Chui   +7 more
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Fetal Hemoglobin, Sickling, and Sickle Cell Disease

Advances in Pediatrics, 1990
Increased numbers of F cells and large amounts of Hb F/F cell appear to produce clinical benefit in rare variants of sickle cell disease and probably in more commonly encountered patients. Fetal hemoglobin interferes with polymerization of Hb S in vitro, but laboratory studies carried out with homogeneous hemoglobin solutions are inadequate models of ...
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Effect of piracetam on sickle erythrocytes and sickle hemoglobin

Biochimica et Biophysica Acta (BBA) - Protein Structure, 1981
Piracetam, 2-oxo-1-pyrrolidine acetamide, inhibits sickling of red cells containing sickle hemoglobin (Hb S). The concentration required for 50% inhibition is about 300 mM. Addition of piracetam into the supersaturated Hb S solution in concentrated phosphate buffer prolongs the delay time prior to gelation.
T, Asakura, S T, Ohnishi, K, Adachi, M, Ozguc, K, Hashimoto, M T, Devlin, E, Schwartz   +6 more
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The interactions of sickle hemoglobin

Biochimie, 1972
Resume Les interactions entre molecules d'hemoglobine qui conduisent a la falciformation des erythrocytes, aux crises micro-emboliques et a l'anemie chronique sont dues a la presence de la desoxyhemoglobine S (α 2 a β 2 6Val ). Au cours de la desoxygenation la viscosite des solutions concentrees d'hemoglobine S augmente progressivement; il y a ...
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Plasma Hemoglobin and Hemoglobin Fractions in Sickle Cell Crisis

American Journal of Clinical Pathology, 1971
The geometric mean of plasma hemoglobin concentrations assayed by a modified benzidine procedure in 14 patients with sickle cell anemia (S-S hemoglobin) during 18 painful crises was 8.5 mg. per 100 ml. as compared with 5.9 mg. per 100 ml. during quiescent periods and 0.3 mg. per 100 ml. in normal controls. On the first and second days of crisis, plasma
H N, Naumann, L W, Diggs, L, Barreras, B J, Williams   +3 more
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Sickle Hemoglobin Fibers: Mechanisms of Depolymerization

Journal of Molecular Biology, 2002
We examined the depolymerization of hemoglobin (Hb) S fibers in the presence of CO by using photolysis of COHbS to create and isolate individual fibers, then removing photolysis to induce depolymerization. Depolymerization occurs at two sites, fiber ends and fiber sides, with different kinetics and by different mechanisms. At low partial pressure of CO
Gunjan, Agarwal, Jiang Cheng, Wang, Suzanna, Kwong, Scott M, Cohen, Frank A, Ferrone, Robert, Josephs, Robin W, Briehl   +6 more
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Intermolecular contacts within sickle hemoglobin fibers

Journal of Molecular Biology, 1989
By combining X-ray crystallographic co-ordinates of sickle hemoglobin (HbS) molecules with three-dimensional reconstructions of electron micrographs of HbS fibers we have synthesized a model for the structure of the clinically relevant HbS fiber. This model largely accounts for the action of 55 point mutations of HbS whose effect on fiber formation has
S J, Watowich, L J, Gross, R, Josephs
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Interactions of Other Hemoglobin Variants with Sickle-Cell Hemoglobin

New England Journal of Medicine, 1970
Deoxygenation-induced sickling of erythrocytes and gelling of hemolysates containing sickle-cell hemoglobin (Hb) are manifestations of the tendency of deoxyhemoglobin S in concentrated solutions to aggregate with the formation of gels or tactoids (liquid crystals).
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Structural analysis of polymers of sickle cell hemoglobin. II. Sickle hemoglobin macrofibers.

Journal of molecular biology, 1988
Sickle cell hemoglobin macrofibers are an important intermediate in the low pH crystallization pathway of deoxygenated hemoglobin S that link the fiber to the crystal. Macrofibers are a class of helical particles differing primarily in their diameters but are related by a common packing of their constituent subunits. We have performed three-dimensional
D A, Bluemke, B, Carragher, M J, Potel, R, Josephs   +3 more
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Helical crystals of sickle cell hemoglobin

Journal of Molecular Biology, 1980
Abstract Thin ribbon-like crystals are intermediates in the formation of large crystals of deoxyhemoglobin S from many individual fibers. The thin crystals show foldedover regions when observed by electron microscopy. Some crystals are sufficiently long to have several folds each separated by a distance of about 4.4 μm, suggesting that the crystals ...
T E, Wellems, R, Josephs
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