刺猬信號通路在人類早期發育中起著重要作用。該通路是一個信號級聯，在發育過程中起到模式形成和細胞增殖的作用。這個基因編碼了刺猬信號通路的負調節因子。這個基因的缺陷是髓母細胞瘤的原因。選擇性剪接導致多個轉錄變體。
The Hedgehog signaling pathway plays an important role in early human development. The pathway is a signaling cascade that plays a role in pattern formation and cellular proliferation during development. This gene encodes a negative regulator of the hedgehog signaling pathway. Defects in this gene are a cause of medulloblastoma. Alternative splicing results in multiple transcript variants.