TRANSFORMING GROWTH-FACTOR-BETA AND MEGAKARYOCYTES IN THE PATHOGENESIS OF IDIOPATHIC MYELOFIBROSIS

TRANSFORMING GROWTH-FACTOR-BETA AND MEGAKARYOCYTES IN THE PATHOGENESIS OF IDIOPATHIC MYELOFIBROSIS
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DOI:
10.1111/j.1365-2141.1994.tb04970.x
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发表时间:
1994-09-01
影响因子:
6.5
通讯作者:
BAUTERS, F
BAUTERS, F
中科院分区:
医学2区
文献类型:
--
作者:
MARTYRE, MC;ROMQUIN, N;BAUTERS, F

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虽然该病已被很好地描述,但特发性骨髓纤维化的发病机制仍不清楚。我们先前报道了与健康受试者相比,这种骨髓增生性疾病患者血小板内转化生长因子-β(TGF-β)水平升高。在这里,在一系列的16名患者中,我们发现TGF-β的表达也在患者的外周血单核细胞(PBMC)中增加:(i)在mRNA水平上通过北方印迹杂交和/或逆转录聚合酶链反应(RT-PCR)分析;(ii)及/或在分泌肽水平,如通过对CC 164细胞的生长抑制测定在来自患者单核细胞的条件培养基中评估的。用多克隆抗TGF-β(1)抗体,TGF-β定位于形态异质性细胞:用gpII(B)III(a)单克隆抗体标记,这些细胞被鉴定为巨核细胞。
Although the disease is well described, the pathogenesis of bone marrow fibrosis in idiopathic myelofibrosis still remains unclear. We previously reported elevated intraplatelet transforming growth factor-beta (TGF-beta) levels in patients with this myeloproliferative disorder, compared with healthy subjects. Here, in a series of 16 patients, we show that TGF-beta expression is also increased in patients' peripheral blood mononuclear cells (PBMC): (i) at the mRNA level analysed by Northern blot hybridization and/or reverse transcription-polymerase chain reaction (RT-PCR); (ii) and/or at the secreted peptide level as evaluated in conditioned media from patients' mononuclear cells by a growth inhibition assay on CC164 cells.By immunostaining with a polyclonal anti-TGF-beta(1) antibody, TGF-P was localized in morphologically heterogenous cells: these cells were characterized as megakaryocytes by labelling with a gpII(b)III(a) monoclonal antibody.Thus we provide evidence that both TGF-beta and megakaryocytes are linked in the pathogenesis of idiopathic myelofibrosis.