2010/09/27 by Jon C Aster, Jon C. Aster, Stephen C Blacklow +3 · 162 citations
Biochemistry, Genetics and Molecular Biology · Immunology and Microbiology · Medicine · #Biology #Cancer research #Cancer-related gene regulation #Epigenetics and DNA Methylation #Hematology #Immune system #Immunology #Lymphoblastic lymphoma #Lymphoma #Medicine #Pathology #T cell #T-cell and Retrovirus Studies
paper · doi:10.1002/path.2789
published in The Journal of Pathology 223(2), 263-274
openalex publication_date 2010/09/27 · openalex created_date 2025/10/10 · openalex updated_date 2026/08/01
Notch receptors participate in a highly conserved signalling pathway that regulates normal development and tissue homeostasis in a context- and dose-dependent manner. Deregulated Notch signalling has been implicated in many diseases, but the clearest example of a pathogenic role is found in T-cell lymphoblastic leukaemia/lymphoma (T-LL), in which the majority of human and murine tumours have acquired mutations that lead to aberrant increases in Notch1 signalling. Remarkably, it appears that the selective pressure for Notch mutations is virtually unique among cancers to T-LL, presumably reflecting a special context-dependent role for Notch in normal T-cell progenitors. Nevertheless, there are some recent reports suggesting that Notch signalling has subtle, yet important roles in other forms of haematological malignancy as well. Here, we review the role of Notch signalling in various blood cancers, focusing on T-LL with an eye towards targeted therapeutics.