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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  <identifier identifierType="URL">https://aperta.ulakbim.gov.tr/record/110672</identifier>
  <creators>
    <creator>
      <creatorName>Cagnan, Ilgin</creatorName>
      <givenName>Ilgin</givenName>
      <familyName>Cagnan</familyName>
      <affiliation>Hacettepe Univ, Inst Hlth Sci, Dept Stem Cell Sci, Ctr Stem Cell Res &amp; Dev, TR-06100 Ankara, Turkey</affiliation>
    </creator>
    <creator>
      <creatorName>Gunel-Ozcan, Aysen</creatorName>
      <givenName>Aysen</givenName>
      <familyName>Gunel-Ozcan</familyName>
      <affiliation>Hacettepe Univ, Inst Hlth Sci, Dept Stem Cell Sci, Ctr Stem Cell Res &amp; Dev, TR-06100 Ankara, Turkey</affiliation>
    </creator>
    <creator>
      <creatorName>Aerts-Kaya, Fatima</creatorName>
      <givenName>Fatima</givenName>
      <familyName>Aerts-Kaya</familyName>
      <affiliation>Hacettepe Univ, Inst Hlth Sci, Dept Stem Cell Sci, Ctr Stem Cell Res &amp; Dev, TR-06100 Ankara, Turkey</affiliation>
    </creator>
    <creator>
      <creatorName>Ameziane, Najim</creatorName>
      <givenName>Najim</givenName>
      <familyName>Ameziane</familyName>
      <affiliation>Vrije Univ Amsterdam Med Ctr, Dept Clin Genet, Amsterdam, Netherlands</affiliation>
    </creator>
    <creator>
      <creatorName>Kuskonmaz, Baris</creatorName>
      <givenName>Baris</givenName>
      <familyName>Kuskonmaz</familyName>
      <affiliation>Hacettepe Univ, Div Bone Marrow Transplantat Unit, Dept Pediat, Fac Med, Ankara, Turkey</affiliation>
    </creator>
    <creator>
      <creatorName>Dorsman, Josephine</creatorName>
      <givenName>Josephine</givenName>
      <familyName>Dorsman</familyName>
      <affiliation>Vrije Univ Amsterdam Med Ctr, Dept Clin Genet, Amsterdam, Netherlands</affiliation>
    </creator>
    <creator>
      <creatorName>Gumruk, Fatma</creatorName>
      <givenName>Fatma</givenName>
      <familyName>Gumruk</familyName>
      <affiliation>Hacettepe Univ, Dept Pediat Hematol, Fac Med, Ankara, Turkey</affiliation>
    </creator>
    <creator>
      <creatorName>Uckan, Duygu</creatorName>
      <givenName>Duygu</givenName>
      <familyName>Uckan</familyName>
    </creator>
  </creators>
  <titles>
    <title>Bone Marrow Mesenchymal Stem Cells Carrying Fancd2 Mutation Differ From The Other Fanconi Anemia Complementation Groups In Terms Of Tgf-Beta 1 Production</title>
  </titles>
  <publisher>Aperta</publisher>
  <publicationYear>2018</publicationYear>
  <dates>
    <date dateType="Issued">2018-01-01</date>
  </dates>
  <resourceType resourceTypeGeneral="Text">Journal article</resourceType>
  <alternateIdentifiers>
    <alternateIdentifier alternateIdentifierType="url">https://aperta.ulakbim.gov.tr/record/110672</alternateIdentifier>
  </alternateIdentifiers>
  <relatedIdentifiers>
    <relatedIdentifier relatedIdentifierType="DOI" relationType="IsIdenticalTo">10.1007/s12015-017-9794-5</relatedIdentifier>
  </relatedIdentifiers>
  <rightsList>
    <rights rightsURI="http://www.opendefinition.org/licenses/cc-by">Creative Commons Attribution</rights>
    <rights rightsURI="info:eu-repo/semantics/openAccess">Open Access</rights>
  </rightsList>
  <descriptions>
    <description descriptionType="Abstract">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.</description>
  </descriptions>
</resource>