Deregulation of immunoglobulin gene transcription in the Hodgkin-Reed Sternberg cell line L1236

Hodgkin-Reed Sternberg (H-RS) cells harbour clonal immunoglobulin gene (Ig) rearrangements in almost all cases of classical Hodgkin's disease but lack Ig gene expression. In the H-RS cell line L1236. a somatic mutation of the Ig heavy-chain gene promoter octamer motif has been described as a putativ e reason for absence of Ig gene expression. We addressed transcriptional ac tivity of this mutated promoter by performing reporter gene studies and gel retardation assays. The results showed that the mutation outside the codin g region of the rearranged Ig gene in L1236 cells leads to downregulation o f Ig gene expression in H-RS cells.