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Perturbation of the transforming growth factor β system in Turner syndrome
Jian Zhou1, Sruthi Arepalli, Clara M Cheng
1National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, MD 20892, USA. zhouj@mail.nih.gov
Objective:
To measure components of the circulating transforming growth factor β (TGFβ) system in patients with Turner syndrome (TS) compared to relevant controls and to ascertain correlation with endocrine and cardiovascular parameters.
Methods:
TGFβ1, TGFβ2 and endoglin (a vascular TGF receptor component) were measured using enzyme-linked immunoassays in the sera of 40 subjects with TS and 40 healthy volunteer women. The cardiovascular phenotype in TS subjects was extensively characterized by cardiac magnetic resonance (MR) and echo.
Results:
TGFβ1 levels were about 3-fold higher in TS while TGFβ2 levels were about 3.5-fold higher in controls (P<0.000 1 for both). Soluble endoglin levels were 25% higher in TS (P<0.000 1). Variation in TGFβ system components was not explained by age, blood pressure, platelet count, thyroid function, body proportions or cardiovascular phenotype.
Conclusion:
There is profound perturbation of the TGFβ system evident in the circulation of individuals with TS.
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