Notch and NF-κB signaling pathways in the biology of classical Hodgkin lymphoma

R Schwarzer, F Jundt - Current molecular medicine, 2011 - ingentaconnect.com
R Schwarzer, F Jundt
Current molecular medicine, 2011ingentaconnect.com
Classical Hodgkin lymphoma (cHL) is now recognized as a B-cell-derived lymphoma which
is characterized by only about 1% malignant pathognomonic Hodgkin and Reed-Sternberg
(HRS) cells and an abundant infiltrate of reactive bystander cells. HRS cells are unique with
respect to their lost B-cell-specific gene expression pattern and recurrent genetic lesions.
Aberrant activity of Notch signaling, a highly conserved developmental pathway, acts as a
negative regulator of the B cell program in HRS cells and thereby contributes to their …
Classical Hodgkin lymphoma (cHL) is now recognized as a B-cell-derived lymphoma which is characterized by only about 1% malignant pathognomonic Hodgkin and Reed-Sternberg (HRS) cells and an abundant infiltrate of reactive bystander cells. HRS cells are unique with respect to their lost B-cell-specific gene expression pattern and recurrent genetic lesions. Aberrant activity of Notch signaling, a highly conserved developmental pathway, acts as a negative regulator of the B cell program in HRS cells and thereby contributes to their reprogramming. Another striking feature and the major pathogenetic mechanism in HRS cells is constitutive NF-κB activation. A number of aberrations that contribute to canonical NF-κB activity in HRS cells have been described such as genetic lesions, deregulated receptor signaling and Epstein-Barr virus (EBV) infection. The importance of Notch and NF-κB signaling for cHL pathogenesis, their potential cross-talk and implications for future therapeutic applications are being discussed.
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