Circulating Transforming Growth Factor <I>β</I>-1 Level in Japanese Patients With Marfan Syndrome
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Marfan syndrome (MFS) is an inherited connective tissue disorder mainly caused by the fibrillin-1 mutation. Deficient fibrillin-1 is thought to result in the failed sequestration of transforming growth factor <I>β</I> (TGF<I>β</I>) and subsequent activation of the TGF<I>β</I> signaling pathway, suggesting that the circulating TGF<I>β</I> level may be elevated in MFS, although its accurate measurement is complex due to <I>ex vivo</I> release from platelet stores upon platelet activation. We measured the plasma TGF<I>β</I>1 levels of 32 Japanese MFS patients (22 medically untreated, 10 treated, 20 males, 30.1 ± 9.6 years old) and 30 healthy volunteers (19 males, 29.5 ± 5.8 years old) by ruthenium-based electrochemiluminescence platform (ECL). PF4 was also measured by enzyme immunoassay (EIA) as a platelet degranulation marker. There was no significant difference in the mean plasma TGF<I>β</I>1 level between the MFS group (1.31 ± 0.40 ng/mL) and controls (1.17 ± 0.33 ng/mL) (<I>P</I> = 0.16, NS). Also, there was no significant difference between the untreated (1.24 ± 0.37 ng/mL) and treated (1.46 ± 0.45 ng/mL) MFS patients (<I>P</I> = 0.15, NS). We also measured PF4, which showed wide deviations but no significant difference between the two groups (<I>P</I> = 0.50). A difference in circulating TGF<I>β</I>1 levels between MFS patients and controls was not detected in this Japanese population. Circulating TGF<I>β</I>1 is not a diagnostic and therapeutic marker for Japanese MFS patients, although our findings do not eliminate the possible association of TGF<I>β</I> with the pathogenesis of MFS.
- International Heart Journal
International Heart Journal 54(1), 23-26, 2013
International Heart Journal Association