The State of Hemoglobin in Sickled Erythrocytes
نویسنده
چکیده
Electron microscopic and other evidence have provided support for the hypothesis that the sickling phenomenon is due to the intracellular formation of long slender crystals of reduced sickle hemoglobin. The rapid growth of these crystals causing tenting of the cell membrane is responsible for the bizarre distortion of the erythrocytes and presumably for the disease itself.
منابع مشابه
The State of Hemoglobin in Sick.led Erythrocytes by Chandler
Sickle cell anemia has been termed a "molecular disease" because of the demonstration that it is due to a genetically determined abnormality of hemoglobin, consisting of a single amino acid substitution in the t-chains of the globin (1-3). Erythrocytes containing sickle hemoglobin possess the usual biconcave discoid shape when the hemoglobin is combined with oxygen, but when oxygen is removed s...
متن کاملThe fine structure of sickled hemoglobin in situ.
E CRUMPLING of disc-shaped erythrocytes into bizarre forms is considered the basic pathological feature of sickle cell anemia.1 Several investigators have established that erythrocyte sickling is due to intracellular precipitation of deoxygenated sickle hemoglobin ( Hb S ).2.T A recent ultrastructural study by Murayarna identified the presence of tubular rods of Hb S in whole mount preparations...
متن کاملThe Physical State of Hemoglobin in Sickle-cell Anemia Erythrocytes in Vivo
Venous blood removed anaerobically from patients with sickle-cell anemia was transferred immediately into fixative, thus precluding significant loss or gain of oxygen by the cells. Electron microscopy demonstrated an intraerythrocytic fibrillar fine structure similar to that described in prior studies on erythrocytes sickled by deoxygenation in vitro. Observations reported here lead to these co...
متن کاملEffects of hypertonic stress on the fine structure of sickled erythrocytes.
U NDER THE INFLUENCE OF DECREASED OXYGEN TENSION or reducing agents, erythrocytes from patients with sickle cell disease develop bizarre contortions of biconcave shape.1 Current concepts of the mechanism involved in this phenomenon are based in part on Harris’s2 investigation of stroma-free solutions of sickled hemoglobin ( HbS ) . He noted formation of spindle-shaped bundles of rod-like elemen...
متن کاملGels of Normal and Sickled Hemoglobin
Electron microscopic studies of the sickling phenomenon have described at least three different structural arrangements of sickled hemoglobin, including empty hexagonal crystals, microtubules, and solid rods. It is unlikely that sickling results in several different polymers, and it is essential to determine the true structure of sickled hemoglobin in order to define the mechanism of molecular ...
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عنوان ژورنال:
- The Journal of Experimental Medicine
دوره 123 شماره
صفحات -
تاریخ انتشار 1966