title: Transforming growth factor-beta and megakaryocytes in the pathogenesis of idiopathic myelofibrosis.
abstract: 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-beta was localized in morphologically heterogenous cells; these cells were characterized as megakaryocytes by labelling with a gpIIbIIIa monoclonal antibody. Thus we provide evidence that both TGF-beta and megakaryocytes are linked in the pathogenesis of idiopathic myelofibrosis.
authors: Unité 365 INSERM, Institut Curie, Section de Biologie, Paris, France.
authors: M C
authors: MC
authors: Martyré
authors: N
authors: N
authors: Romquin
authors: M C
authors: MC
authors: Le Bousse-Kerdiles
authors: S
authors: S
authors: Chevillard
authors: B
authors: B
authors: Benyahia
authors: B
authors: B
authors: Dupriez
authors: J L
authors: JL
authors: Demory
authors: F
authors: F
authors: Bauters
mesh_terms: D000368:Aged; D001483:Base Sequence; D015152:Blotting, Northern; D005260:Female; D015870:Gene Expression; D006801:Humans; D008297:Male; D008533:Megakaryocytes / Q000378:metabolism* / Q000473:pathology; D008875:Middle Aged; D008969:Molecular Sequence Data; D009000:Monocytes / Q000378:metabolism; D016133:Polymerase Chain Reaction; D055728:Primary Myelofibrosis / Q000209:etiology* / Q000235:genetics / Q000473:pathology; D016212:Transforming Growth Factor beta / Q000235:genetics / Q000378:metabolism*
chemical_list: D016212:Transforming Growth Factor beta