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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.

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