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Sökning: WFRF:(Demoulin JB)

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  • Classen, Jean-Francois, et al. (författare)
  • Lack of evidence of stimulatory autoantibodies to platelet-derived growth factor receptor in patients with systemic sclerosis
  • 2009
  • Ingår i: Arthritis and Rheumatism. - 0004-3591 .- 1529-0131. ; 60:4, s. 1137-1144
  • Tidskriftsartikel (refereegranskat)abstract
    • OBJECTIVE: Systemic sclerosis (SSc) is a severe connective tissue disease of unknown etiology, characterized by fibrosis of the skin and multiple internal organs. Recent findings suggested that the disease is driven by stimulatory autoantibodies to platelet-derived growth factor receptor (PDGFR), which stimulate the production of reactive oxygen species (ROS) and collagen by fibroblasts. These results opened novel avenues of research into the diagnosis and treatment of SSc. The present study was undertaken to confirm the presence of anti-PDGFR antibodies in patients with SSc. METHODS: Immunoglobulins from 37 patients with SSc were purified by protein A/G chromatography. PDGFR activation was tested using 4 different sensitive bioassays, i.e., cell proliferation, ROS production, signal transduction, and receptor phosphorylation; the latter was also tested in a separate population of 7 patients with SSc from a different research center. RESULTS: Purified IgG samples from patients with SSc were positive when tested for antinuclear autoantibodies, but did not specifically activate PDGFRalpha or PDGFRbeta in any of the tests. Cell stimulation with PDGF itself consistently produced a strong signal. CONCLUSION: The present results raise questions regarding the existence of agonistic autoantibodies to PDGFR in SSc.
  • Demoulin, Jean-Baptiste, et al. (författare)
  • Scleroderma
  • 2009
  • Ingår i: New England Journal of Medicine. - 0028-4793 .- 1533-4406. ; 361:8, s. 826-827
  • Tidskriftsartikel (refereegranskat)abstract
    • In their review article on scleroderma, Gabrielli et al. incorporate their earlier observations that all patients with this disease have activating antibodies to platelet-derived growth factor (PDGF) receptors (PDGFRs). These antibodies were described as being specific for scleroderma and for chronic extensive graft-versus-host disease, two conditions that share some clinical features. The review does not reflect the current debate on the existence of such antibodies. Researchers from several centers, including ours, have challenged the results of Gabrielli et al.
  • Velghe, AI, et al. (författare)
  • PDGFRA alterations in cancer : characterization of a gain-of-function V536E transmembrane mutant as well as loss-of-function and passenger mutations
  • 2014
  • Ingår i: Oncogene. - : Nature Publishing Group. - 0950-9232 .- 1476-5594. ; 33:20, s. 2568-2576
  • Tidskriftsartikel (refereegranskat)abstract
    • Activating mutations in the platelet-derived growth factor (PDGF) receptor alpha (PDGFRA) have been described in patients with gastrointestinal stromal tumors or myeloid malignancies associated with hypereosinophilia. These patients respond well to imatinib mesylate, raising the question as to whether patients with a PDGF receptor mutation in other tumor types should receive a tyrosine kinase inhibitor treatment. We characterized 10 novel somatic point mutations in PDGFRA that have been reported in isolated cases of glioblastoma, melanoma, acute myeloid leukemia, peripheral nerve sheath tumors and neuroendocrine carcinoma. The PDGFRA transmembrane domain mutation V536E stimulated Ba/F3 cell growth and signaling via ERK and STAT5 in the absence of ligand. This mutant, identified in glioblastoma, was strongly inhibited by imatinib. Modeling suggested that the mutation modulates the packing of the transmembrane domain helices in the receptor dimer. By contrast, two mutations in highly conserved residues affected the receptor traffic to the cell surface or kinase activity, thereby preventing the response to PDGF. The other mutations had no significant impact on the receptor activity. This functional analysis matched the predictions of SIFT and PolyPhen for only five mutations and these algorithms do not discriminate gain from loss of function. Finally, an E996K variant that had been identified in a melanoma cell line was not expressed in these cells. Altogether, several newly identified PDGFRA mutations do not activate the receptor and may therefore represent passenger mutations. Our results also underline the importance of characterizing novel kinase alterations in cancer patients.
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