Ektacytometry
Mostrando 1-7 de 7 artigos, teses e dissertações.
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1. Evaluation of the red cell deformability through ektacytometry in iron deficiency / Avaliação da deformidade eritrocitária através da ectacitometria na deficiência de ferro
The deformability allows the 7 to 8 cm red cell to circulate through capillaries of 3 cm. This phenomenon depends on cellular geometry, internal viscosity and viscoelastic properties of the membrane. Among the various techniques of erytrocyte deformability (ED) analysis such as: micropipette aspiration, filtration and reoscopy, we chose ektacytometry. This t
Publicado em: 2002
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2. Alport syndrome, mental retardation, midface hypoplasia, and elliptocytosis: a new X linked contiguous gene deletion syndrome?
We describe a family with four members, a mother, two sons, and a daughter, who show clinical features consistent with X linked Alport syndrome. The two males presented with additional features including mental retardation, dysmorphic facies with marked midface hypoplasia, and elliptocytosis. The elliptocytosis was not associated with any detectable abnormal
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3. Removal of erythrocyte membrane iron in vivo ameliorates the pathobiology of murine thalassemia.
Abnormal deposits of free iron are found on the cytoplasmic surface of red blood cell (RBC) membranes in beta-thalassemia. To test the hypothesis that this is of importance to RBC pathobiology, we administered the iron chelator deferiprone (L1) intraperitoneally to beta-thalassemic mice for 4 wk and then studied RBC survival and membrane characteristics. L1
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4. Mild spherocytosis and altered red cell ion transport in protein 4.2–null mice
Protein 4.2 is a major component of the red blood cell (RBC) membrane skeleton. We used targeted mutagenesis in embryonic stem (ES) cells to elucidate protein 4.2 functions in vivo. Protein 4.2–null (4.2–/–) mice have mild hereditary spherocytosis (HS). Scanning electron microscopy and ektacytometry confirm loss of membrane surface in 4.2–/– RBCs.
American Society for Clinical Investigation.
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5. Oral magnesium supplements reduce erythrocyte dehydration in patients with sickle cell disease.
Intracellular polymerization and sickling depend markedly on the cellular concentration of sickle hemoglobin (Hb S). A possible therapeutic strategy for sickle cell disease is based on reducing the cellular concentration of Hb S through prevention of erythrocyte dehydration. The K-Cl cotransporter is a major determinant of sickle cell dehydration and is inhi
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6. Headpiece domain of dematin is required for the stability of the erythrocyte membrane
Dematin is an actin-binding and bundling protein of the erythrocyte membrane skeleton. Dematin is localized to the spectrin–actin junctions, and its actin-bundling activity is regulated by phosphorylation of cAMP-dependent protein kinase. The carboxyl terminus of dematin is homologous to the “headpiece” domain of villin, an actin-bundling protein of th
The National Academy of Sciences.
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7. Molecular and functional changes in spectrin from patients with hereditary pyropoikilocytosis.
The structural and functional properties of spectrin from normal and hereditary pyropoikilocytosis (HPP) donors from the two unrelated families were studied. The structural domains of the spectrin molecule were generated by mild tryptic digestion and analyzed by two-dimensional electrophoresis (isoelectric focusing; sodium dodecyl sulfate-polyacrylamide gel