GLI1是致癌基因，是一种锌指转录因子，通过调节Sonic hedgehog (Shh)信号通路而在正常的神经发育和功能中发挥重要作用。GLI1转录因子直接结合DNA激活Shh靶基因来响应通路激活，并且可以根据信号背景而作为一种转录激活子和转录抑制子发挥作用。在没有Shh配体的情况下，GLI1由蛋白酶裂解以产生对转录激活所必需的缺乏C-端氨基酸的截短蛋白。继Shh配体暴露后，GLI1不再裂解，并能激活Shh靶基因的转录。GLI1是一种致癌基因，通常在胶质母细胞瘤中发生突变或过表达，这可能导致Shh-GLI信号通路的异常激活。

GLI1 is a zinc-finger transcription factor that plays an important role in normal neural development and function via regulation of the Sonic hedgehog (Shh) signaling pathway. The GLI1 transcription factor binds DNA directly to activate Shh target genes in response to pathway activation and can function as both a transcription activator and transcription repressor depending on the signaling context. In the absence of Shh ligand, GLI1 is cleaved by proteases to produce a truncated protein lacking C-terminal amino acids necessary for transcription activation. Following Shh ligand exposure, GLI1 is no longer cleaved and can activate transcription of Shh target genes. GLI1 is an oncogene and is typically mutated or overexpressed in glioblastomas, where it can cause aberrant activation of the Shh–Gli signaling pathway.

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