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首页> 外文期刊>Cytokine >Evaluation of the bioactive and total transforming growth factor beta1 levels in primary myelofibrosis.
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Evaluation of the bioactive and total transforming growth factor beta1 levels in primary myelofibrosis.

机译:评价原发性骨髓纤维化的生物活性和总转化生长因子β1水平。

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TGFbeta1 is secreted as latent protein that requires activation to become biologically active. It negatively regulates the progenitor cell growth, and favours the deposition of extra-cellular matrix in different tissues. We have studied TGFbeta1 levels in Philadelphia-negative (Ph-) myeloproliferative diseases, evaluating patients with primary myelofibrosis (PMF) that is characterized by increased numbers of circulating progenitor cells and bone marrow (BM) fibrosis, and patients with polycythemia vera (PV) or essential thrombocythemia (ET) that do not present BM fibrosis. We found that patients with PMF, PV or ET have higher peripheral blood (PB) plasma levels of both bioactive and total TGFbeta1 than healthy controls, with a balance bioactive/total TGFbeta1 in favour of the latter. The balance between bioactive/total TGFbeta1 in the BM plasma of patients mirrored that of PB, with most of TGFbeta1 in the latent form; on the contrary, in the BM plasma of healthy controls most of the TGFbeta1 was in the bioactive form. In conclusion, increased plasma levels of TGFbeta1 and an altered ratio bioactive/total TGFbeta1 in BM are not peculiar of patients with PMF suggesting that, whether altered levels of TGFbeta1 have a role in myelofibrosis, this may not be related to the induction of BM fibrosis.
机译:TGFbeta1作为潜在蛋白分泌,需要激活才能具有生物活性。它负面调节祖细胞的生长,并促进细胞外基质在不同组织中的沉积。我们已经研究了费城阴性(Ph-)骨髓增生性疾病中的TGFbeta1水平,评估了以循环祖细胞和骨髓(BM)纤维化数目增加为特征的原发性骨髓纤维化(PMF)患者和真性红细胞增多症(PV)患者或不出现BM纤维化的原发性血小板增多症(ET)。我们发现,与健康对照组相比,PMF,PV或ET患者的外周血(PB)血浆中的生物活性和总TGFbeta1均较高,而生物活性/总TGFbeta1则相对于健康对照组更高。患者的BM血浆中的生物活性/总TGFbeta1之间的平衡反映了PB的平衡,其中大部分TGFbeta1为潜伏形式。相反,在健康对照组的BM血浆中,大多数TGFbeta1处于生物活性形式。总之,血浆PMB患者血浆TGFbeta1水平升高和生物活性/总TGFbeta1比例改变并非PMF患者所特有,这表明,TGFbeta1水平改变是否与骨髓纤维化有关,这可能与诱导BM纤维化无关。

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