scholarly journals Red cell membrane and cation deficiency in Rh null syndrome

Blood ◽  
1984 ◽  
Vol 63 (5) ◽  
pp. 1046-1055 ◽  
Author(s):  
SK Ballas ◽  
MR Clark ◽  
N Mohandas ◽  
HF Colfer ◽  
MS Caswell ◽  
...  

Abstract A 52-yr-old multiparous white female was found to have Rh null blood type. She had macrocytic anemia, with reticulocytosis (15%-20%), of long duration. Although stomatocytes in peripheral blood were numerous and osmotic fragility was increased, suggesting increased cell water, the RBC cation content, and thus cell water, was decreased. Cell dehydration was confirmed by an increased proportion of high density RBC on Stractan density gradients. The deformability of RBC from four gradient subpopulations was measured in the ektacytometer as a function of suspending medium osmolality. Analysis of these measurements showed an abnormal reduction in cell surface area with increasing cell density, thus explaining the increased osmotic fragility of whole blood. This was confirmed by a density-dependent reduction in cell cholesterol content, suggesting membrane instability in vivo. Rh null subpopulations showed a twofold increase in both ouabain-sensitive and - insensitive Na-K ATPase activity and 86Rb transport, even in the dense fraction with the fewest reticulocytes. No membrane protein or glycoprotein abnormality was detected by SDS-PAGE. The associated deficiencies of both membrane surface area and cation content in Rh null cells, as well as increased Na-K pump activity, suggest a pleiotropic functional interrelationship among Rh antigen, membrane stability, and cation regulation.

Blood ◽  
1984 ◽  
Vol 63 (5) ◽  
pp. 1046-1055 ◽  
Author(s):  
SK Ballas ◽  
MR Clark ◽  
N Mohandas ◽  
HF Colfer ◽  
MS Caswell ◽  
...  

A 52-yr-old multiparous white female was found to have Rh null blood type. She had macrocytic anemia, with reticulocytosis (15%-20%), of long duration. Although stomatocytes in peripheral blood were numerous and osmotic fragility was increased, suggesting increased cell water, the RBC cation content, and thus cell water, was decreased. Cell dehydration was confirmed by an increased proportion of high density RBC on Stractan density gradients. The deformability of RBC from four gradient subpopulations was measured in the ektacytometer as a function of suspending medium osmolality. Analysis of these measurements showed an abnormal reduction in cell surface area with increasing cell density, thus explaining the increased osmotic fragility of whole blood. This was confirmed by a density-dependent reduction in cell cholesterol content, suggesting membrane instability in vivo. Rh null subpopulations showed a twofold increase in both ouabain-sensitive and - insensitive Na-K ATPase activity and 86Rb transport, even in the dense fraction with the fewest reticulocytes. No membrane protein or glycoprotein abnormality was detected by SDS-PAGE. The associated deficiencies of both membrane surface area and cation content in Rh null cells, as well as increased Na-K pump activity, suggest a pleiotropic functional interrelationship among Rh antigen, membrane stability, and cation regulation.


Blood ◽  
1983 ◽  
Vol 61 (5) ◽  
pp. 899-910 ◽  
Author(s):  
MR Clark ◽  
N Mohandas ◽  
SB Shohet

Whole cell deformability of red cells was measured as a continuous function of suspending medium osmolality using the ektacytometer, a laser-diffraction viscometer. Study of normal cells in which water content and membrane surface area had been selectively modified showed that this technique can detect changes in these properties with high sensitivity. The osmotic deformability profiles obtained from this assay provide information about cell water content, surface area, and the heterogeneity in these cellular properties, information that by conventional methods would require several different types of measurements. Application of this approach to a variety of pathologic blood samples showed that various hematologic disorders can be characterized by the shape of this profile and the position of specific features of the profile along the osmolality axis. Measurement of osmotic deformability profiles thus provides a convenient and comprehensive means of identifying abnormalities either in red cell water content or surface area.


Blood ◽  
1983 ◽  
Vol 61 (5) ◽  
pp. 899-910 ◽  
Author(s):  
MR Clark ◽  
N Mohandas ◽  
SB Shohet

Abstract Whole cell deformability of red cells was measured as a continuous function of suspending medium osmolality using the ektacytometer, a laser-diffraction viscometer. Study of normal cells in which water content and membrane surface area had been selectively modified showed that this technique can detect changes in these properties with high sensitivity. The osmotic deformability profiles obtained from this assay provide information about cell water content, surface area, and the heterogeneity in these cellular properties, information that by conventional methods would require several different types of measurements. Application of this approach to a variety of pathologic blood samples showed that various hematologic disorders can be characterized by the shape of this profile and the position of specific features of the profile along the osmolality axis. Measurement of osmotic deformability profiles thus provides a convenient and comprehensive means of identifying abnormalities either in red cell water content or surface area.


Blood ◽  
1975 ◽  
Vol 46 (3) ◽  
pp. 337-356 ◽  
Author(s):  
JS Wiley ◽  
JC Ellory ◽  
MA Shuman ◽  
CC Shaller ◽  
RA Cooper

Cation permeability and lipid composition have been studied in the red cells of five patients with various features of the hereditary stomatocytosis syndrome. Hemolysis was compensated in four patients, and only one patient was anemic. Cell NA+ was increased an average of 3 mueq per ml cells and cell K+ decreased 14 mueq per ml cells. Both active and passive fluxes of Na+ and K+ were increased by two to six times normal. Tritiated ouabain binding was increased an average of 2.5- fold, suggesting a proportionally greater number of cation pumps per cell. The coupling ratio of active Na+:K+ fluxes was normal (3:2). Calcium permeability was increased compatible with the degree of reticulocytosis, and cell Ca2+ content was normal. The lowered sum of Na+ plus K+ was associated with a high MCHC and low cell water. When examined in wet preparations, red cells assumed either a bowl-shaped or an irregular contour, and they appeared as target cells on dry smears. Only when cell water was increased in hypotonic media were stomatocytes seen on smear. The total lipid content of red cells was increased in four patients, although it was normal in one. The mole ratio of cholesterol to phospholipid was always normal; however, phospholipid analysis showed an increased proportion of phosphatidyl choline. The abnormal cells were osmotically resistant due to both an increased membrane surface area and a low total cation content. These patients show two hallmarks of hereditary stomatocytosis: bowlshaped red cells observed on wet preparations and a marked increase in Na+ and K+ permeability. The heterogeneity of this syndrome in our patients and in others reported with hereditary stomatocytosis appears to result from (1) variability in the increase in surface area which results from an excess of membrane lipid content, particularly phosphatidylcholine, and (2) a variability in cell water content which may be either decreased or increased as a result of changes in the sum of Na+ plus K+ ions.


Blood ◽  
1975 ◽  
Vol 46 (3) ◽  
pp. 337-356 ◽  
Author(s):  
JS Wiley ◽  
JC Ellory ◽  
MA Shuman ◽  
CC Shaller ◽  
RA Cooper

Abstract Cation permeability and lipid composition have been studied in the red cells of five patients with various features of the hereditary stomatocytosis syndrome. Hemolysis was compensated in four patients, and only one patient was anemic. Cell NA+ was increased an average of 3 mueq per ml cells and cell K+ decreased 14 mueq per ml cells. Both active and passive fluxes of Na+ and K+ were increased by two to six times normal. Tritiated ouabain binding was increased an average of 2.5- fold, suggesting a proportionally greater number of cation pumps per cell. The coupling ratio of active Na+:K+ fluxes was normal (3:2). Calcium permeability was increased compatible with the degree of reticulocytosis, and cell Ca2+ content was normal. The lowered sum of Na+ plus K+ was associated with a high MCHC and low cell water. When examined in wet preparations, red cells assumed either a bowl-shaped or an irregular contour, and they appeared as target cells on dry smears. Only when cell water was increased in hypotonic media were stomatocytes seen on smear. The total lipid content of red cells was increased in four patients, although it was normal in one. The mole ratio of cholesterol to phospholipid was always normal; however, phospholipid analysis showed an increased proportion of phosphatidyl choline. The abnormal cells were osmotically resistant due to both an increased membrane surface area and a low total cation content. These patients show two hallmarks of hereditary stomatocytosis: bowlshaped red cells observed on wet preparations and a marked increase in Na+ and K+ permeability. The heterogeneity of this syndrome in our patients and in others reported with hereditary stomatocytosis appears to result from (1) variability in the increase in surface area which results from an excess of membrane lipid content, particularly phosphatidylcholine, and (2) a variability in cell water content which may be either decreased or increased as a result of changes in the sum of Na+ plus K+ ions.


Blood ◽  
2008 ◽  
Vol 112 (11) ◽  
pp. 2874-2874
Author(s):  
Luigia De Falco ◽  
Lucia De Franceschi ◽  
Frank Borgese ◽  
Carmelo Piscopo ◽  
Maria Rosaria Esposito ◽  
...  

Abstract Stomatocytosis is an inherited autosomal dominant hemolytic anemia and includes overhydrated hereditary stomatocytosis (OHS), dehydrated hereditary stomatocytosis (DHS), hereditary cryohydrocytosis (CHC) and familial pseudohyperkalemia (FP). Here, we report a novel variant of hereditary stomatocytosis due to a de-novo band 3 mutation due to G>A transition at nucleotide 2500 in exon 17 (p. G796R, band3CEINGE) associated with dyserythropoietic phenotype. This 43-years-old Caucasian female (II-2) with unrelated parents was admitted to our hospital for mild anemia evaluation. The patient was in good health until 7 years when she frequently experienced asthenia. Anemia was first recognized at the age of eighth years with presence of jaundice and hyperchromic urine, but she had never received blood transfusions. We observed a mild hypochromic macrocytic anemia with a hemoglobin level of 11.5 g/dL, a mean cell volume (MCV) of 110 fL, and a mean hemoglobin concentration (MCH) of 36.1 pg, the reticulocyte count was 64 × 103/μL. There was a typical hemolytic features: high levels of indirect bilirubin (3.48 mg/dL) and lactate dehydrogenase ( 567 U/l, v.n. 240– 480 U/l ) with negativity at direct and indirect Coomb’s test. Spleen was enlarged and ultrasonography detected 15 cm of longitudinal size. She was cholecystectomized at the age of 14 years because of numerous symptomatic small stones. Serum iron, soluble transferrin receptor, serum ferritin and transferrin saturation levels were all increased, while the transferrin was in the normal range.Other blood tests including osmotic fragility with incubated and fresh erythrocytes, serum electrolytes, B12 and folate levels, erythrocyte enzyme levels, EMA test and Pink test were normal. Peripheral blood smear showed anisopoikilocytosis with rare stomatocytes and no spherocytes. Bone marrow aspirate showed remarkable dyserythropoiesis with increased number of erythroblasts and binucleate erythroblasts, basophilic erythroblasts with alterations, irregular nuclei maturation, intererythroblastic bridges and erythroblasts with basophilic stippling. She received since the age of 14 yrs a diagnosis for congenital dyserythropoietic anemia type I. Patients red cells showed increase Na+ content and decrease K+ content; reduced Na-K pump activity and increased Na-H exchange, NKCC cotransport and KCC cotransport activities. We then functionally characterized band 3 CEINGE in Xenopus oocytes, showing that the mutated band 3 is converted from anion exchanger (Cl−, HCO3 −) function to unregulated cation pathway for Na+ and K+. The mutated band 3 was also associated with increased tyrosine phosphorylation pattern of some red cell membrane proteins. During erythropoiesis band 3 protein is the last cytoskeletal protein to appear, thus the dyserythropoietic phenotype may be related to a possible role of the mutated band 3 in perturbation of cytoskeleton assembly in the late stage of erythropoiesis, allowing us to conclude for a new variant of stomatocytosis with dyserythropoietic phenotype.


Author(s):  
M. G. Markova ◽  
E. N. Somova

Work on going through the adaptation stage of rooted micro-stalks comes down to searching for new growth regulators and studying the influence of external conditions, which include, among other things, light effects. The data of 2018-2019 on the effect of growth regulators Siliplant, EcoFus and experimental LED phytoradiators on the adaptation of rooted micro-stalks of garden strawberries (Fragaria x ananassa duch) in vivo are presented. The object of research is rooted micro-stalks of garden strawberries of the Korona variety. It was revealed that, at the adaptation stage of rooted micro-stalks of strawberries, the most effective was the treatment of plants by spraying with Siliplant at a concentration of 1.0 ml/l and the combined treatment with Siliplant and EcoFus at concentrations of 0.5 ml/l: regardless of lighting, the survival rate averaged 99.4 - 99.7%, the leaf surface area increased significantly from 291.85 mm2 to 334.4 mm2. The number of normally developed leaves of strawberry microplants increased significantly after treatment with all preparations from 3.5 to 6.0, 5.8 and 6.5 pcs/plant, and a significant increase in the height of strawberry rosettes was facilitated by treatment with Siliplant and Siliplant together with EcoFus. Regardless of growth regulators, the most effective was the experimental LED phyto-irradiator with a changing spectrum, which contributed to an increase in leaf surface area, height of rosettes and the number of normally developed leaves in strawberry microplants. When illuminated with a flashing phytoradiator, these indicators are lower than in the control version, but not significantly. By the end of the rooting stage, all microplants of garden strawberries corresponded to GOST R 54051-2010.


2021 ◽  
Vol 89 (2) ◽  
pp. 15
Author(s):  
M. R. Mozafari ◽  
E. Mazaheri ◽  
K. Dormiani

Introduction: Bioactive encapsulation and drug delivery systems have already found their way to the market as efficient therapeutics to combat infections, viral diseases and different types of cancer. The fields of food fortification, nutraceutical supplementation and cosmeceuticals have also been getting the benefit of encapsulation technologies. Aim: Successful formulation of such therapeutic and nutraceutical compounds requires thorough analysis and assessment of certain characteristics including particle number and surface area without the need to employ sophisticated analytical techniques. Solution: Here we present simple mathematical formulas and equations used in the research and development of drug delivery and controlled release systems employed for bioactive encapsulation and targeting the sites of infection and cancer in vitro and in vivo. Systems covered in this entry include lipidic vesicles, polymeric capsules, metallic particles as well as surfactant- and tocopherol-based micro- and nanocarriers.


Author(s):  
Alessio Facciolà ◽  
Giuseppa Visalli ◽  
Marianna Pruiti Ciarello ◽  
Angela Di Pietro

Plastics are ubiquitous persistent pollutants, forming the most representative material of the Anthropocene. In the environment, they undergo wear and tear (i.e., mechanical fragmentation, and slow photo and thermo-oxidative degradation) forming secondary microplastics (MPs). Further fragmentation of primary and secondary MPs results in nanoplastics (NPs). To assess potential health damage due to human exposure to airborne MPs and NPs, we summarize the evidence collected to date that, however, has almost completely focused on monitoring and the effects of airborne MPs. Only in vivo and in vitro studies have assessed the toxicity of NPs, and a standardized method for their analysis in environmental matrices is still missing. The main sources of indoor and outdoor exposure to these pollutants include synthetic textile fibers, rubber tires, upholstery and household furniture, and landfills. Although both MPs and NPs can reach the alveolar surface, the latter can pass into the bloodstream, overcoming the pulmonary epithelial barrier. Despite the low reactivity, the number of surface area atoms per unit mass is high in MPs and NPs, greatly enhancing the surface area for chemical reactions with bodily fluids and tissue in direct contact. This is proven in polyvinyl chloride (PVC) and flock workers, who are prone to persistent inflammatory stimulation, leading to pulmonary fibrosis or even carcinogenesis.


1992 ◽  
Vol 101 (4) ◽  
pp. 907-913 ◽  
Author(s):  
G.J. Cannon ◽  
J.A. Swanson

Murine bone marrow-derived macrophages, which measure 13.8 +/− 2.3 microns diameter in suspension, can ingest IgG-opsonized latex beads greater than 20 microns diameter. A precise assay has allowed the determination of the phagocytic capacity, and of physiological parameters that limit that capacity. Ingestion of beads larger than 15 microns diameter required IgG-opsonization, and took 30 minutes to reach completion. Despite the dependence on Fc-receptors for phagocytosis of larger beads, cells reached their limit before all cell surface Fc-receptors were occupied. The maximal membrane surface area after frustrated phagocytosis of opsonized coverslips was similar to the membrane surface area required to engulf particles at the limiting diameter, indicating that the capacity was independent of particle shape. Vacuolation of the lysosomal compartment with sucrose, which expanded endocytic compartments, lowered the phagocytic capacity. This decrease was reversed when sucrose vacuoles were collapsed by incubation of cells with invertase. These experiments indicate that the phagocytic capacity is limited by the amount of available membrane, rather than by the availability of Fc-receptors. The capacity was also reduced by depolymerization of cytoplasmic microtubules with nocodazole. Nocodazole did not affect the area of maximal cell spreading during frustrated phagocytosis, but did alter the shape of the spread cells. Thus, microtubules may coordinate cytoplasm for engulfment of the largest particles.


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