Can genes normally expressed only in the eye be activated in brain tumours? Such a phenomenon, though surprising, has been observed in certain types of medulloblastoma, paediatric tumours of the cerebellum. Researchers from the CNRS, Institut Curie, Inserm and Université Paris-Sud(1), together with researchers at St. Jude Children’s Research Hospital (Memphis, United States), have pinpointed the role of these genes in the tumour process, thus offering new therapeutic targets. Their findings appear in the 12 March 2018 edition of Cancer Cell.
Medulloblastoma is treated with a combination of surgery, radiotherapy and chemotherapy, which results in a survival rate of 80 %, albeit with significant side effects. Group 3 medulloblastoma, associated with frequent rates of recurrence and a much lower survival rate, is characterised by the expression of a gene cluster named “photoreceptor program”. Normally, these genes are only expressed in the retina, where they define photoreceptor identity and, in particular, ensure that light signals are converted into nerve impulses.
Given that they are not expressed during the normal development of the cerebellum, activation of these genes in medulloblastoma is very surprising. Aberrant differentiation programs – unrelated to the tissue in which the tumour originates – have already been found
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