Analysis of mesenchymal cells derived from an chondrodysplasia punctuate patient and donors

Journal of Cellular Biochemistry
Irena ShurDafna Benayahu

Abstract

Conradi-Hunermann syndrome (CDPX2) is X-linked dominant disorder appeared with aberrant punctuate calcification. The skeletal cells derived from the marrow stroma are active in maintaining the skeletal formation. We obtained mesenchymal stem cells from a patient with CDPX2 and studied the formation of colony forming unit-fibroblast (CFU-F) in vitro in comparison cells obtained from normal donors. Cultured cells were studied morphologically and subjected to gene expression analysis. Marrow stromal cells (MSC)-chondrodysplasia punctuate (CDP) cells from CDPX2 were identified by their mosaic morphology formed three phenotypically distinct types of CFU-F colonies. One type consisted of normal fibroblasts with developed cell body and cellular processes; the second type contained pathological small cells without processes; and the third type comprised of mixed cells. We compared gene expression by the MSC-CDP to cells from normal donors. Transcription factors analyzed proliferation potential were similar in both normal and mixed colonies of MSC-CDP and similar to normal MSCs. The message expression for cytokines and extra cellular matrix (ECM) proteins revealed similar expression for biglycan, osteocalcin, and osteonectin, while IL-6...Continue Reading

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Sep 5, 2002·Journal of Cellular Biochemistry·I ShurD Benayahu

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Citations

May 9, 2008·Journal of Molecular and Cellular Cardiology·Caroline SteingenWilhelm Bloch
Jul 11, 2006·Stem Cells·Annette SchmidtWilhelm Bloch
Jul 11, 2006·European Journal of Cell Biology·Annette SchmidtWilhelm Bloch

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