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Relationship of glutathione levels and Heinz body formation to irreversibly sickled cells in sickle cell anemia.
J Lab Clin Med. 1984 Apr; 103(4):589-96.JL

Abstract

To assess the possibility that the membrane lesion seen in sickle cells involves oxidant damage, we investigated the relationship between irreversibly sickled cells and glutathione levels and Heinz body production. We found that the mean level of red cell glutathione, whether expressed as micromoles per gram of hemoglobin or micromoles per milliliter of cell water, was significantly decreased in 39 patients with sickle cell anemia. A weak but significant negative correlation between glutathione levels and irreversibly sickled cell counts in the patients was observed. Stractan density gradient studies were performed to determine glutathione differences in normal- and abnormal-appearing sickle cells. The percent of abnormal cells in each of the five stractan gradient fractions was negatively correlated with both the micromole per gram of hemoglobin and micromole per milliliter of cell water levels of glutathione. Another indicator of susceptibility to oxidant stress, Heinz body formation, was increased in patients with sickle cell anemia vs controls in response to oxidant stress. Surprisingly, we found that the majority of Heinz bodies were in the non-ISC fraction of cells, rather than in the irreversibly sickled cells.

Authors

Wetterstroem N
No affiliation info available
Brewer GJ
No affiliation info available
Warth JA
No affiliation info available
Mitchinson A
No affiliation info available
Near K
No affiliation info available

MeSH

Anemia, Sickle CellErythrocytes, AbnormalGlucosephosphate Dehydrogenase DeficiencyGlutathioneHeinz BodiesHumansRegression Analysis

Pub Type(s)

Journal Article
Research Support, Non-U.S. Gov't
Research Support, U.S. Gov't, P.H.S.

Language

eng

PubMed ID

6699474

Citation

Wetterstroem, N, et al. "Relationship of Glutathione Levels and Heinz Body Formation to Irreversibly Sickled Cells in Sickle Cell Anemia." The Journal of Laboratory and Clinical Medicine, vol. 103, no. 4, 1984, pp. 589-96.
Wetterstroem N, Brewer GJ, Warth JA, et al. Relationship of glutathione levels and Heinz body formation to irreversibly sickled cells in sickle cell anemia. J Lab Clin Med. 1984;103(4):589-96.
Wetterstroem, N., Brewer, G. J., Warth, J. A., Mitchinson, A., & Near, K. (1984). Relationship of glutathione levels and Heinz body formation to irreversibly sickled cells in sickle cell anemia. The Journal of Laboratory and Clinical Medicine, 103(4), 589-96.
Wetterstroem N, et al. Relationship of Glutathione Levels and Heinz Body Formation to Irreversibly Sickled Cells in Sickle Cell Anemia. J Lab Clin Med. 1984;103(4):589-96. PubMed PMID: 6699474.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Relationship of glutathione levels and Heinz body formation to irreversibly sickled cells in sickle cell anemia. AU - Wetterstroem,N, AU - Brewer,G J, AU - Warth,J A, AU - Mitchinson,A, AU - Near,K, PY - 1984/4/1/pubmed PY - 1984/4/1/medline PY - 1984/4/1/entrez SP - 589 EP - 96 JF - The Journal of laboratory and clinical medicine JO - J Lab Clin Med VL - 103 IS - 4 N2 - To assess the possibility that the membrane lesion seen in sickle cells involves oxidant damage, we investigated the relationship between irreversibly sickled cells and glutathione levels and Heinz body production. We found that the mean level of red cell glutathione, whether expressed as micromoles per gram of hemoglobin or micromoles per milliliter of cell water, was significantly decreased in 39 patients with sickle cell anemia. A weak but significant negative correlation between glutathione levels and irreversibly sickled cell counts in the patients was observed. Stractan density gradient studies were performed to determine glutathione differences in normal- and abnormal-appearing sickle cells. The percent of abnormal cells in each of the five stractan gradient fractions was negatively correlated with both the micromole per gram of hemoglobin and micromole per milliliter of cell water levels of glutathione. Another indicator of susceptibility to oxidant stress, Heinz body formation, was increased in patients with sickle cell anemia vs controls in response to oxidant stress. Surprisingly, we found that the majority of Heinz bodies were in the non-ISC fraction of cells, rather than in the irreversibly sickled cells. SN - 0022-2143 UR - https://www.unboundmedicine.com/medline/citation/6699474/Relationship_of_glutathione_levels_and_Heinz_body_formation_to_irreversibly_sickled_cells_in_sickle_cell_anemia_ L2 - http://babysfirsttest.org/newborn-screening/conditions/sickle-cell-anemia DB - PRIME DP - Unbound Medicine ER -