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Blood, 15 April 2002, Vol. 99, No. 8, pp. 3060-3062

BRIEF REPORT

Loss of PU.1 expression is associated with defective immunoglobulin transcription in Hodgkin and Reed-Sternberg cells of classical Hodgkin disease

Franziska Jundt, Katharina Kley, Ioannis Anagnostopoulos, Kristina Schulze Pröbsting, Axel Greiner, Stephan Mathas, Claus Scheidereit, Thomas Wirth, Harald Stein, and Bernd Dörken

From the Charité, Robert-Rössle-Klinik, Humboldt University of Berlin, Germany; Max Delbrück Center for Molecular Medicine, Berlin, Germany; Institute of Pathology, Klinikum Benjamin Franklin, Free University of Berlin, Germany; Institute of Pathology, University of Würzburg, Germany; Department of Physiological Chemistry, University of Ulm, Germany

Immunoglobulin transcription is impaired in Hodgkin and Reed-Sternberg (HRS) cells of classical Hodgkin disease (cHD). We recently demonstrated that defective immunoglobulin promoter transcription correlates with the down-regulation of the B-cell transcription factors Oct2 and BOB.1/OBF.1. These results prompted us to investigate whether immunoglobulin enhancer activity is also impaired in HRS cells and whether as yet unidentified factors could be necessary for immunoglobulin enhancer activity in HRS cells of cHD. Here we analyzed 30 cases of cHD for expression of the Ets family member PU.1 that is known to collaborate with multiple transcription factors and to regulate expression of immunoglobulin genes. We show that PU.1 is not expressed in primary and cultured HRS cells. Reintroduction of PU.1 and Oct2 in cultured HRS cells restored the activity of cotransduced immunoglobulin enhancer constructs. Our study identifies PU.1 deficiency as a recurrent defect in HRS cells that might contribute to their impairment of immunoglobulin transcription.

© 2002 by The American Society of Hematology.
 

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