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Abnormality of erythrocyte membrane n-3 long chain polyunsaturated fatty acids in sickle cell haemoglobin C (HbSC) disease is not as remarkable as in sickle cell anaemia (HbSS).

Abstract

Sickle cell disease (SCD) is a group of inherited blood disorders in which clinical illness results from the presence of erythrocytes with sickled haemoglobin (HbS). Blood vessel occlusion is a fundamental pathological process in SCD. Sickle cell haemoglobin C (HbSC) disease and sickle cell anaemia (HbSS) share some pathophysiology and clinical manifestations. However, the former is generally less severe. Erythrocytes of HbSC patients have longer life span, reduced haemolysis, and lower propensity to adhere to vascular endothelium than those of their HbSS counterparts. The structure and function of erythrocytes are strongly modulated by membrane long chain polyunsaturated fatty acids (LCPUFA). We have tested the possibility that HbSC and HbSS patients have different membrane fatty acid composition consistent with the difference in their clinical severity. Steady-state patients, 9 HbSC and 28 HbSS, and 15 HbAA were studied. The HbSC patients had a higher level of linoleic (LA, P<0.05) and docosahexaenoic (DHA, P<0.05) acids, and lower arachidonic acid (AA, P<0.01) and AA/eicosapentaenoic acid (EPA) ratio (P<0.05) in erythrocyte choline phosphoglycerides (CPG) compared with the HbSS group. Similarly, the level of EPA was higher and AA/EPA ratio (P<0.01) lower in serine phosphoglycerides of the HbSC patients. In contrast to the HbSC, the HbSS group had lower levels of EPA (P<0.001), DHA (P<0.05), total n-3 metabolites and total n-3 fatty acids (P<0.001) in erythrocyte CPG compared with the healthy HbAA controls. Moreover, the HbSS patients with disease complications compared with those without complications had reduced DHA and total n-3 fatty acids (P<0.005) in erythrocyte CPG. The abnormalities in erythrocyte in LCPUFA which is manifested by an increase in AA and a decrease in EPA and DHA in HbSS relative to HbSC disease observed in this study are consistent with the contrast in clinical severity between the two entities.

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  • Authors+Show Affiliations

    ,

    Institute of Brain Chemistry and Human Nutrition, London Metropolitan University, London, UK. Hor016@londonmet.ac.uk

    , , , ,

    Source

    MeSH

    Adult
    Aged
    Anemia, Sickle Cell
    Erythrocyte Membrane
    Erythrocytes
    Fatty Acids, Omega-3
    Fatty Acids, Unsaturated
    Hemoglobin SC Disease
    Hemoglobins
    Humans
    Leukocyte Count
    Middle Aged
    Phosphatidylcholines
    Phosphatidylethanolamines
    Phosphatidylserines
    Triglycerides

    Pub Type(s)

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

    Language

    eng

    PubMed ID

    16314081

    Citation

    Ren, Hongmei, et al. "Abnormality of Erythrocyte Membrane N-3 Long Chain Polyunsaturated Fatty Acids in Sickle Cell Haemoglobin C (HbSC) Disease Is Not as Remarkable as in Sickle Cell Anaemia (HbSS)." Prostaglandins, Leukotrienes, and Essential Fatty Acids, vol. 74, no. 1, 2006, pp. 1-6.
    Ren H, Ghebremeskel K, Okpala I, et al. Abnormality of erythrocyte membrane n-3 long chain polyunsaturated fatty acids in sickle cell haemoglobin C (HbSC) disease is not as remarkable as in sickle cell anaemia (HbSS). Prostaglandins Leukot Essent Fatty Acids. 2006;74(1):1-6.
    Ren, H., Ghebremeskel, K., Okpala, I., Ugochukwu, C. C., Crawford, M., & Ibegbulam, O. (2006). Abnormality of erythrocyte membrane n-3 long chain polyunsaturated fatty acids in sickle cell haemoglobin C (HbSC) disease is not as remarkable as in sickle cell anaemia (HbSS). Prostaglandins, Leukotrienes, and Essential Fatty Acids, 74(1), pp. 1-6.
    Ren H, et al. Abnormality of Erythrocyte Membrane N-3 Long Chain Polyunsaturated Fatty Acids in Sickle Cell Haemoglobin C (HbSC) Disease Is Not as Remarkable as in Sickle Cell Anaemia (HbSS). Prostaglandins Leukot Essent Fatty Acids. 2006;74(1):1-6. PubMed PMID: 16314081.
    * Article titles in AMA citation format should be in sentence-case
    TY - JOUR T1 - Abnormality of erythrocyte membrane n-3 long chain polyunsaturated fatty acids in sickle cell haemoglobin C (HbSC) disease is not as remarkable as in sickle cell anaemia (HbSS). AU - Ren,Hongmei, AU - Ghebremeskel,Kebreab, AU - Okpala,Iheanyi, AU - Ugochukwu,Cynthia C, AU - Crawford,Michael, AU - Ibegbulam,Obike, Y1 - 2005/11/28/ PY - 2005/07/06/received PY - 2005/10/11/revised PY - 2005/10/16/accepted PY - 2005/11/30/pubmed PY - 2006/2/28/medline PY - 2005/11/30/entrez SP - 1 EP - 6 JF - Prostaglandins, leukotrienes, and essential fatty acids JO - Prostaglandins Leukot. Essent. Fatty Acids VL - 74 IS - 1 N2 - Sickle cell disease (SCD) is a group of inherited blood disorders in which clinical illness results from the presence of erythrocytes with sickled haemoglobin (HbS). Blood vessel occlusion is a fundamental pathological process in SCD. Sickle cell haemoglobin C (HbSC) disease and sickle cell anaemia (HbSS) share some pathophysiology and clinical manifestations. However, the former is generally less severe. Erythrocytes of HbSC patients have longer life span, reduced haemolysis, and lower propensity to adhere to vascular endothelium than those of their HbSS counterparts. The structure and function of erythrocytes are strongly modulated by membrane long chain polyunsaturated fatty acids (LCPUFA). We have tested the possibility that HbSC and HbSS patients have different membrane fatty acid composition consistent with the difference in their clinical severity. Steady-state patients, 9 HbSC and 28 HbSS, and 15 HbAA were studied. The HbSC patients had a higher level of linoleic (LA, P<0.05) and docosahexaenoic (DHA, P<0.05) acids, and lower arachidonic acid (AA, P<0.01) and AA/eicosapentaenoic acid (EPA) ratio (P<0.05) in erythrocyte choline phosphoglycerides (CPG) compared with the HbSS group. Similarly, the level of EPA was higher and AA/EPA ratio (P<0.01) lower in serine phosphoglycerides of the HbSC patients. In contrast to the HbSC, the HbSS group had lower levels of EPA (P<0.001), DHA (P<0.05), total n-3 metabolites and total n-3 fatty acids (P<0.001) in erythrocyte CPG compared with the healthy HbAA controls. Moreover, the HbSS patients with disease complications compared with those without complications had reduced DHA and total n-3 fatty acids (P<0.005) in erythrocyte CPG. The abnormalities in erythrocyte in LCPUFA which is manifested by an increase in AA and a decrease in EPA and DHA in HbSS relative to HbSC disease observed in this study are consistent with the contrast in clinical severity between the two entities. SN - 0952-3278 UR - https://www.unboundmedicine.com/medline/citation/16314081/Abnormality_of_erythrocyte_membrane_n_3_long_chain_polyunsaturated_fatty_acids_in_sickle_cell_haemoglobin_C__HbSC__disease_is_not_as_remarkable_as_in_sickle_cell_anaemia__HbSS__ L2 - https://linkinghub.elsevier.com/retrieve/pii/S0952-3278(05)00174-2 DB - PRIME DP - Unbound Medicine ER -