1. Evidence for a proatherogenic biochemical phenotype in beta thalassemia minor and intermedia.
- Author
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Lai ME, Vacquer S, Carta MP, Spiga A, Cocco P, Abete C, Dessì S, and Mandas A
- Subjects
- Acetyl-CoA C-Acetyltransferase genetics, Acetyl-CoA C-Acetyltransferase metabolism, Adult, Antimicrobial Cationic Peptides blood, Antimicrobial Cationic Peptides genetics, Antimicrobial Cationic Peptides metabolism, Atherosclerosis epidemiology, Cholesterol, HDL blood, Erythropoietin blood, Female, Hepcidins, Humans, Interleukin-1alpha genetics, Interleukin-1alpha metabolism, Iron analysis, Iron blood, Italy epidemiology, Leukocytes, Mononuclear metabolism, Male, Middle Aged, Oxidative Stress, Phenotype, RNA, Messenger metabolism, Risk Factors, Severity of Illness Index, Sterol Esterase genetics, Sterol Esterase metabolism, Transferrin chemistry, Transferrin metabolism, Tumor Necrosis Factor-alpha genetics, Tumor Necrosis Factor-alpha metabolism, beta-Thalassemia blood, beta-Thalassemia metabolism, Atherosclerosis etiology, beta-Thalassemia physiopathology
- Abstract
The purpose of this study was to focus on pathophysiological mechanisms linking β-thalassemia intermedia (β-TI) and minor (β-TMI) with cardiovascular risk. Iron status, prooxidant-antioxidant balance and lipid profiles in serum, and lipid content in peripheral blood mononuclear cells (PBMCs) were evaluated in 20 β-TMI subjects, 22 β-TI patients and in 30 nonthalassemic blood donors. The mRNA levels of some genes involved in the regulation of iron and cholesterol metabolism were also determined. In β-TI and in β-TMI, serum iron, prooxidant-antioxidant ratio, transferrin saturation and erythropoietin levels were higher, while transferrin and hepcidin were lower compared to controls. Hepcidin and interleukin-1α mRNA levels were found to be reduced in β-TI- and β-TMI-PBMCs, while those of tumor necrosis factor alpha were increased. A reduction in high-density lipoprotein cholesterol in serum and an accumulation of neutral lipids coupled with increased mRNA levels of acetyl-coenzyme A:cholesterol acyltransferase and decreased neutral cholesterol ester hydrolase in PBMCs were also observed in β-TI and β-TMI compared to controls. Taken together, these findings provide experimental support for the idea that not only β-TI patients but also β-TMI have a proatherogenic biochemical phenotype which may contribute to increase their cardiovascular disease risk., (Copyright © 2011 S. Karger AG, Basel.)
- Published
- 2011
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