{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,6]],"date-time":"2026-10-06T07:23:30Z","timestamp":1791271410966,"version":"4.1.0"},"reference-count":57,"publisher":"Wiley","issue":"8","license":[{"start":{"date-parts":[[2006,3,31]],"date-time":"2006-03-31T00:00:00Z","timestamp":1143763200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["The FEBS Journal"],"published-print":{"date-parts":[[2006,4]]},"abstract":"<jats:p>Waardenburg syndrome (WS) is an auditory\u2013pigmentary disorder that exhibits varying combinations of sensorineural hearing loss and abnormal pigmentation of the hair and skin. WS type 4 (WS4), a subtype of WS, is characterized by the presence of the aganglionic megacolon and is associated with mutations in the gene encoding either endothelin 3, endothelin receptor type B (EDNRB), or Sry\u2010box 10 (SOX10). Here, we provide evidence that SOX10 regulates the expression of <jats:italic>EDNRB<\/jats:italic> gene in human melanocyte\u2010lineage cells, as judged by RNA interference and chromatin immunoprecipitation analyses. Human melanocytes preferentially express the <jats:italic>EDNRB<\/jats:italic> transcripts derived from the conventional <jats:italic>EDNRB<\/jats:italic> promoter. SOX10 transactivates the <jats:italic>EDNRB<\/jats:italic> promoter through the <jats:italic>cis<\/jats:italic>\u2010acting elements, the two CA\u2010rich sequences and the GC box. Moreover, a transcription factor Sp1 enhances the degree of the SOX10\u2010mediated transactivation of the <jats:italic>EDNRB<\/jats:italic> promoter through these <jats:italic>cis<\/jats:italic>\u2010acting elements. Furthermore, we have shown that the <jats:italic>EDNRB<\/jats:italic> promoter is heavily methylated in HeLa human cervical cancer cells, lacking <jats:italic>EDNRB<\/jats:italic> expression, but not in melanocytes and HMV\u2010II melanoma cells. The expression of <jats:italic>EDNRB<\/jats:italic> became detectable in HeLa cells after treatment with a demethylating reagent, 5\u2032\u2010aza\u20102\u2032\u2010deoxycytidine, which was further enhanced in the transformed cells over\u2010expressing SOX10. We therefore suggest that SOX10, alone or in combination with Sp1, regulates transcription of the <jats:italic>EDNRB<\/jats:italic> gene, thereby ensuring appropriate expression level of EDNRB in human melanocytes.<\/jats:p>","DOI":"10.1111\/j.1742-4658.2006.05200.x","type":"journal-article","created":{"date-parts":[[2006,3,31]],"date-time":"2006-03-31T16:53:04Z","timestamp":1143823984000},"page":"1805-1820","source":"Crossref","is-referenced-by-count":27,"title":["SOX10, in combination with Sp1, regulates the <i>endothelin receptor type B<\/i> gene in human melanocyte lineage 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