Abstract
Ten healthy donors and four patients with Diamond-Blackfan anemia (DBA) have been investigated for granulocyte-macrophage colony stimulating factor (GM-CSF) and stem cell factor (SCF) production by bone marrow-enriched fibroblasts (BMEF) in a highly sensitive biological assay on growth factor-dependent M-07e cells. M-07e cells detected active soluble kit-ligand from normal bone marrow fibroblasts as well as from DBA BMEF which produce constitutively significant amounts of SCF. Interleukin 1 beta (IL-1 beta) induced a significant increase of soluble SCF from both normal and DBA BMEF. GM-CSF was undetectable in unstimulated cultures, while its production by bone marrow microenvironmental cells was documented for both normal and DBA patients after IL-1 beta stimulation in vitro.
Publication types
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Research Support, Non-U.S. Gov't
MeSH terms
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Adult
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Biological Assay*
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Bone Marrow / metabolism*
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Bone Marrow / pathology
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Cells, Cultured / drug effects
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Child
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Child, Preschool
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Fanconi Anemia / metabolism*
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Fanconi Anemia / pathology
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Female
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Fibroblasts / metabolism*
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Fibroblasts / pathology
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Gene Expression
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Granulocyte-Macrophage Colony-Stimulating Factor / analysis
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Granulocyte-Macrophage Colony-Stimulating Factor / biosynthesis*
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Granulocyte-Macrophage Colony-Stimulating Factor / pharmacology
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Hematopoietic Cell Growth Factors / analysis
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Hematopoietic Cell Growth Factors / biosynthesis*
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Hematopoietic Cell Growth Factors / pharmacology
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Hematopoietic Stem Cells / drug effects*
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Humans
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Interleukin-1 / pharmacology
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Male
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RNA, Messenger / genetics
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Stem Cell Factor
Substances
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Hematopoietic Cell Growth Factors
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Interleukin-1
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RNA, Messenger
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Stem Cell Factor
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Granulocyte-Macrophage Colony-Stimulating Factor