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Hoffmann, R. A wiki for the life sciences where authorship matters. Nature Genetics (2008)

The reduction of cholesteryl linoleate in lipoproteins: an index of clinical severity in beta-thalassemia/Hb E.

BACKGROUND: Oxidative modification of lipoproteins has been reported in beta-thalassemia and has been suggested to relate to atherogenesis-risk. This study focused on the change in cholesteryl esters in plasma lipoproteins under oxidative stress resulting from iron overload in beta-thalassemia/hemoglobin E (beta-thal/Hb E) patients. METHODS: Markers of oxidative damage and cholesteryl esters (CEs) were measured in plasma and lipo-proteins from 30 beta-thal/Hb E patients and compared to those from 10 healthy volunteers. CEs in plasma, low-density lipoprotein (LDL) and high-density lipoprotein (HDL) were separated and identified using HPLC. RESULTS: beta-Thal/Hb E patients presented iron overload, a precipitous decrease in alpha-tocopherol and increased lipid peroxidation (thiobarbituric acid-reactive substances; TBARs) in both plasma and lipoproteins. Cholesteryl linoleate, the most abundant CE in lipoproteins, showed a reduction of 70% in LDL, while other CEs showed a lower reduction (50%). An inverse relationship between the cholesteryl linoleate/cholesteryl oleate ratio (CL/CO) and the degree of clinical severity suggested that the CL/CO ratio is an index of damaged lipoproteins and could be used as a pathologic marker of underlying iron overload. Good correlation of non-transferrin-bound iron (NTBI) and TBARs (r=0.8, p<0.01) in LDL strongly supported the contention that iron overload is responsible for initiating the lipid peroxidation in beta-thal/Hb E. CONCLUSIONS: This study suggests that cholesteryl linoleate is the primary target of oxidative modification induced by NTBI in beta-thal/Hb E patients and that reduction in cholesteryl linoleate in lipoproteins could be used as a severity index for beta-thal/Hb E.[1]


  1. The reduction of cholesteryl linoleate in lipoproteins: an index of clinical severity in beta-thalassemia/Hb E. Luechapudiporn, R., Morales, N.P., Fucharoen, S., Chantharaksri, U. Clin. Chem. Lab. Med. (2006) [Pubmed]
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