{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,11,27]],"date-time":"2025-11-27T14:59:18Z","timestamp":1764255558800,"version":"3.46.0"},"reference-count":38,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2009,1,13]],"date-time":"2009-01-13T00:00:00Z","timestamp":1231804800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["J of Cellular Biochemistry"],"published-print":{"date-parts":[[2009,3]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>Mutation in fibrillin\u20102, a major structural component of extracellular microfibrils in connective tissue, results in the autosomal dominant disease congenital contractural arachnodactyly. This genetic disease is characterized by dolichostenomelia and arachnodactyly, in addition to contractures of the large joints and abnormal pinnae formation, thus indicating the significance of fibrillin\u20102 in chondrogenesis. In this study, we investigated the transcriptional regulation of fibrillin\u20102 in chondrogenic differentiation. Although mRNA expression of fibrillin\u20101, a highly homologous protein to fibrillin\u20102, remained almost unchanged during chondrogenesis of mouse ATDC5 cells, fibrillin\u20102 mRNA expression varied. Fibrillin\u20102 was highly expressed at the early stage and declined progressively during differentiation. The 5\u2032\u2010flanking region of the fibrillin\u20102 gene contains potential binding sites for E2F, Runx, AP\u20102, and Sox transcription factors. The promoter activity of fibrillin\u20102 decreased markedly following deletion and mutagenesis of the E2F binding site between \u2212143 and \u2212136 bp. Overexpression of E2F1 resulted in a marked increase in its promoter activity, whereas expression of other transcription factors including AP\u20102\u03b1 and Runx2 had no effect. The increase in promoter activity by E2F1 was completely suppressed by the coexpression of E2F4. E2F2 and E2F3 had positive effects on the promoter activity. Although ATDC5 cells expressed transcripts for the E2F family genes at all stages of differentiation, the expression profiles differed. E2F1 expression remained almost unchanged, whereas E2F4 expression increased markedly at the late stage of differentiation. These results indicated that coordinated expression of the E2F family is critical for the transcriptional regulation of fibrillin\u20102 during chondrogenesis. J. Cell. Biochem. 106: 580\u2013588, 2009. \u00a9 2009 Wiley\u2010Liss, Inc.<\/jats:p>","DOI":"10.1002\/jcb.22029","type":"journal-article","created":{"date-parts":[[2009,1,13]],"date-time":"2009-01-13T09:22:22Z","timestamp":1231838542000},"page":"580-588","source":"Crossref","is-referenced-by-count":6,"title":["Transcriptional regulation of fibrillin\u20102 gene by E2F family members in chondrocyte 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