Increased Cell Membrane Permeability in the Pathogenesis of Hereditary Spherocytosis *

Jacob, Harry S.; Jandl, James H.; Bell, Sarah Beth; Files, Nancye

doi:10.1172/jci105046

Cited by 160 publications

(85 citation statements)

References 37 publications

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“…This matter is dealt with in greater detail elsewhere [8]. Our findings, however, are consistent with previous observations [1,4,6] which suggest that sodium permeability is increased in the erythrocyte membrane in patients with HS.…”

Section: Discussionsupporting

confidence: 92%

“…These observations on normal subjects arc consistent with the studies of Wiley [11], who reported recently that there was no correlation between sodium influx (presumably measuring permeability) and erythrocyte survival in patients with HS. These findings are inconsistent with the thesis that the increased sodium permeability of erythrocytes of patients with HS plays a central role in the pathogenesis of the hemolytic anemia in this disease [6]. It is more likely the case that the increased sodium permeability characteristic of HS merely reflects an erythrocyte membrane defect in this particular disease.…”

Section: Discussioncontrasting

confidence: 69%

“…In hereditary splierocytosis (HS) the erythrocyte membrane is abnormally permeable to sodium [1,4,6,8]; this tlefcct has been suggested as being responsible for the shortened erythrocyte life span in IIS [6]. In this study, we present evidence contrary to this theory by describing several subjects with little or no evidence of IIS in whom erythrocyte sodium transport is identical to that found in this condition.…”

Section: Introductionmentioning

confidence: 75%

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Significance of Erythrocyte Sodium Flux in the Pathophysiology and Genetic Expression of Hereditary Spherocytosis

Zipursky

Israels

1971

Pediatr Res

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ExtractErythrocyte sodium transport was studied in five families of patients with hereditary spheroeytosis (HS). Values for ouabain-insensitive sodium efilux in the group with HS (mean = 19.9% of cellular sodium-22/hr, so = ±2.7) were significantly greater (P < 0.001) than those found in the normal group (mean = 13.8%, SD = ±2.7). No clinical evidence of HS was found in the parents; however, 4 of the 10 parents had ouabain-insensitive erythrocyte sodium-22 efilux rates in the HS range in comparison to 3 of 30 control subjects, a statistically significant difference (x ! = 4.67; P < 0.05). Speculation

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Section: Discussionsupporting

confidence: 92%

Section: Discussioncontrasting

confidence: 69%

Section: Introductionmentioning

confidence: 75%

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Significance of Erythrocyte Sodium Flux in the Pathophysiology and Genetic Expression of Hereditary Spherocytosis

Zipursky

Israels

1971

Pediatr Res

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“…tially as described by Jacob and Jandl [11]. Whole blood (15-20 ml) was incubated at 37° for 1-2 hr with 10 jxCi of sodium chloride- 22 Na [30].…”

Section: Z2mentioning

confidence: 99%

A New Familial Disorder with Abnormal Erythrocyte Morphology and Increased Permeability of the Erythrocytes to Sodium and Potassium

1971

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ExtractA newborn infant of Philippine parents was found to have a morphological abnormality of his erythrocytes consisting of an elliptical shape of the cells and one or more transverse slitlike areas of decreased density. These changes were also present in erythrocytes of the patient's father, a half-sister of the father, and four of the patient's six siblings. None of the affected family members had anemia or evidence of abnormal hemolysis, and erythrocyte survival by the radiochromium method was normal in three of the individuals studied. Erythrocytes from the affected family members had an increased degree of autohemolysis after incubation for 48 hr, but this was prevented almost entirely by addition of glucose. Glucose consumption in vitro by erythrocytes of the propositus occurred at a rate approximately 60% greater than that of normal controls. The intracellular sodium concentration of the erythrocytes was not different from that of erythrocytes from normal individuals, but a moderate decrease in intracellular potassium was found. When washed cells were incubated in a glucosefree medium, sodium gain and potassium loss were significantly greater than from cells of normal controls. When compared with normal values, efflux of radiosodium was increased during incubation of cells in glucose-containing medium. Erythrocytes from the affected individuals had decreased osmotic fragility, and osmometric measurements indicated a lesser degree of cell swelling in hypotonic solutions than occurred with cells from normal controls. Speculation

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“…It has been reported that, in vitamin E deficient rats, peroxidation of lipid components of red blood cell membrane caused by H2O2 involves the decrease of content of phosphatidylethanolamine and phosphatidyl serine (10,7), and in human cases of abetalipo proteinemia (7) such peroxidation involves the decrease of content of unsaturated fatty acid. In hereditary spherocytosis, although abnormal lipid content in the red blood cell membrane has not been confirmed by most investigators (11,12,13), the relationship between the acce lerated active transport of sodium ion and the acceleration of turnover of phospholipids in hereditary spherocytosis has been noted by Hokin and others (14,15) and Jacob and others (16,17). Therefore, the relationship between accelerated turnover of phospholipids and lipid peroxidation caused by H2O2 in red blood cells of hereditary spherocytosis is considered to be worthy of investigation as a possible cause of elevated H2O2 hemolysis in this anemia.…”

mentioning

confidence: 97%

Hydrogen Peroxide Hemolysis Values and Serum Vitamin E Levels in Various Anemias in Adults

1972

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Increased Cell Membrane Permeability in the Pathogenesis of Hereditary Spherocytosis *

Cited by 160 publications

References 37 publications

Significance of Erythrocyte Sodium Flux in the Pathophysiology and Genetic Expression of Hereditary Spherocytosis

Significance of Erythrocyte Sodium Flux in the Pathophysiology and Genetic Expression of Hereditary Spherocytosis

A New Familial Disorder with Abnormal Erythrocyte Morphology and Increased Permeability of the Erythrocytes to Sodium and Potassium

Hydrogen Peroxide Hemolysis Values and Serum Vitamin E Levels in Various Anemias in Adults

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