1 Ocak 2018 Dergi makalesi Açık Erişim

Cagnan, Ilgin; Gunel-Ozcan, Aysen; Aerts-Kaya, Fatima; Ameziane, Najim; Kuskonmaz, Baris; Dorsman, Josephine; Gumruk, Fatma; Uckan, Duygu

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{
  "@context": "https://schema.org/", 
  "@id": 110672, 
  "@type": "ScholarlyArticle", 
  "creator": [
    {
      "@type": "Person", 
      "affiliation": "Hacettepe Univ, Inst Hlth Sci, Dept Stem Cell Sci, Ctr Stem Cell Res & Dev, TR-06100 Ankara, Turkey", 
      "name": "Cagnan, Ilgin"
    }, 
    {
      "@type": "Person", 
      "affiliation": "Hacettepe Univ, Inst Hlth Sci, Dept Stem Cell Sci, Ctr Stem Cell Res & Dev, TR-06100 Ankara, Turkey", 
      "name": "Gunel-Ozcan, Aysen"
    }, 
    {
      "@type": "Person", 
      "affiliation": "Hacettepe Univ, Inst Hlth Sci, Dept Stem Cell Sci, Ctr Stem Cell Res & Dev, TR-06100 Ankara, Turkey", 
      "name": "Aerts-Kaya, Fatima"
    }, 
    {
      "@type": "Person", 
      "affiliation": "Vrije Univ Amsterdam Med Ctr, Dept Clin Genet, Amsterdam, Netherlands", 
      "name": "Ameziane, Najim"
    }, 
    {
      "@type": "Person", 
      "affiliation": "Hacettepe Univ, Div Bone Marrow Transplantat Unit, Dept Pediat, Fac Med, Ankara, Turkey", 
      "name": "Kuskonmaz, Baris"
    }, 
    {
      "@type": "Person", 
      "affiliation": "Vrije Univ Amsterdam Med Ctr, Dept Clin Genet, Amsterdam, Netherlands", 
      "name": "Dorsman, Josephine"
    }, 
    {
      "@type": "Person", 
      "affiliation": "Hacettepe Univ, Dept Pediat Hematol, Fac Med, Ankara, Turkey", 
      "name": "Gumruk, Fatma"
    }, 
    {
      "@type": "Person", 
      "name": "Uckan, Duygu"
    }
  ], 
  "datePublished": "2018-01-01", 
  "description": "Transforming growth factor beta (TGF-beta) secretion from cells in the bone marrow (BM) niche affects hematopoietic stem cell (HSC) fate and has a cardinal role in HSC quiescence. BM mesenchymal stem cells (BM-MSCs), a component of the BM niche, may produce abnormal levels of TGF-beta in Fanconi anemia (FA) and may play a role in bone marrow failure. Here, we molecularly and cellularly characterized FA BM-MSCs by addressing their immunophenotype, proliferation- and differentiation- capacity, reactive oxygen species (ROS) production, senescence activity as well as expression and secretion levels of TGF-beta isoforms. In ten FA patients, mutations were detected in FANCA (n = 7), FANCG (n = 1) and FANCD2 (n = 2) genes. The immunophenotype, with the exception of CD29, and differentiation capacity of FA BM-MSCs were similar to healthy donors. FA BM-MSCs showed decreased proliferation, increased ROS level and an arrest in G2 following DEB treatment. beta-galactosidase staining indicated elevated senescence of FANCD2-deficient cells. FA BM-MSCs displayed TGF-beta 1 mRNA levels similar to donor BM-MSCs, and was not affected by DEB treatment. However, secretion of TGF-beta was absent in FA-D2 BM-MSCs. Absence of TGF-beta secretion may be related to early onset of senescence of the FANCD2-deficient BM-MSCs. The proliferative response of FA-D2 BM-MSCs to rTGF-beta 1 was not different from FANCA-deficient and donor cells and raises the possibility that rTGF-beta 1 may reverse the senescence of the FANCD2-deficient BM-MSCs which needs to be investigated further.", 
  "headline": "Bone Marrow Mesenchymal Stem Cells Carrying FANCD2 Mutation Differ from the Other Fanconi Anemia Complementation Groups in Terms of TGF-beta 1 Production", 
  "identifier": 110672, 
  "image": "https://aperta.ulakbim.gov.tr/static/img/logo/aperta_logo_with_icon.svg", 
  "license": "http://www.opendefinition.org/licenses/cc-by", 
  "name": "Bone Marrow Mesenchymal Stem Cells Carrying FANCD2 Mutation Differ from the Other Fanconi Anemia Complementation Groups in Terms of TGF-beta 1 Production", 
  "url": "https://aperta.ulakbim.gov.tr/record/110672"
}