Orthodenticle homeobox 2 (OTX2) belongs to the bicoid subfamily of paired-box, homeodomain-containing transcription factors. OTX2 is a critically important neuronal transcription factor that functions to regulate the expression of cell cycle genes controlling proliferation and differentiation of neural progenitor cells . In addition to its neuronal development functions, it has been reported that OTX2 can function in a non-cell autonomous manner to promote survival of damaged retinal ganglion cells . OTX2 has also been shown to influence the susceptibility of post-mitotic neurons to toxic insult or physiological stress . Notably, aberrant expression of OTX2 has been strongly linked with neuronal tumor development. For example, research studies have found OTX2 is overexpressed in many medulloblastoma cell lines, and both overexpression and gene amplification were reported in a subset of primary medulloblastomas . In vitro studies support these observations, as targeted alterations in OTX2 expression directly affected both proliferation and senescence of medulloblastoma cell lines .