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Journal List > J Clin Invest > v.46(3); Mar 1967

Formats:
J Clin Invest. 1967 March; 46(3): 357–368.
doi: 10.1172/JCI105537.
PMCID: PMC297056
Copyright notice
Peroxidative Hemolysis of Red Blood Cells from Patients with Abetalipoproteinemia (Acanthocytosis)*
James T. Dodge,† Gerald Cohen, Herbert J. Kayden,‡ and Gerald B. Phillips§
Department of Medicine, College of Physicians and Surgeons, Columbia University, The New York State Psychiatric Institute, New York, N. Y.
Department of Biochemistry, College of Physicians and Surgeons, Columbia University, The New York State Psychiatric Institute, New York, N. Y.
Department of Psychiatry, College of Physicians and Surgeons, Columbia University, The New York State Psychiatric Institute, New York, N. Y.
Department of Medicine, New York University School of Medicine, New York, N. Y.
† This work was done during the tenure of an Advanced Research Fellowship of the American Heart Association.
‡ Recipient of U. S. Public Health Service Career Research Development Award K3 HE-14-828.
§ Recipient of Lederle Medical Faculty Award. Address requests for reprints to Dr. Gerald B. Phillips, Roosevelt Hospital, 428 W. 59th St., New York, N. Y. 10019.
* Submitted for publication July 1, 1966: accepted November 10, 1966.
This investigation was supported by U. S. Public Health Service grants HE-02907, HE-01045, and HE-06481.
A preliminary report was published in abstract form (1).
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Abstract
The effect of peroxidative stress on tissue was studied by exposure of red blood cells (RBC) from patients with abetalipoproteinemia to minute amounts of H2O2in vitro. Red blood cells from untreated patients showed a marked sensitivity to H2O2, as evidenced by hemolysis and lipid peroxidation (peroxidative hemolysis).
The appearance of lipid peroxidation products in sensitive cells after exposure to H2O2 was indicated by 1) increases in the 2-thiobarbituric acid (TBA) reaction of trichloroacetic acid extracts, 2) increases in ultraviolet light absorbency of lipid extracts, and 3) decreases in polyunsaturated fatty acids. These changes were accompanied by a decrease in phosphatidyl ethanolamine and phosphatidyl serine in the RBC lipid extract. Similar lipid changes on exposure to H2O2 were observed in the RBC from vitamin E-deficient rats.
Treatment of the patients with d-α-tocopherol polyethylene glycol succinate by mouth, or addition of dl-α-tocopherol to the incubation medium protected the RBC from peroxidative hemolysis. Tocopherol appears to provide a primary biologic defense against peroxidative hemolysis.
The presence of nitrite or carbon monoxide, which produced methemoglobin and carboxyhemoglobin, respectively, inhibited peroxidative changes, suggesting a catalytic role for oxy- or deoxyhemoglobin.
Substances that prevented lipid peroxidation also prevented hemolysis; in addition, lipid peroxidation appeared to precede hemolysis. These observations suggested that hemolysis was a consequence of lipid peroxidation.
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Selected References
These references are in PubMed. This may not be the complete list of references from this article.
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