Decreased FOXJ1 expression and its ciliogenesis programme in aggressive ependymoma and choroid plexus tumours

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• 作者：Malak S Abedalthagafi ; Michael P Wu ; Parker H Merrill ; Ziming Du ; Terri Woo ; Shu-Hsien Sheu ; Shelley Hurwitz ; Keith L Ligon and Sandro Santagata
• 关键词：FOXJ1 ; ependymoma ; choroid plexus ; dedifferentiation ; stem cells ; subventricular zone ; cilia ; ciliogenesis
• 刊名：The Journal of Pathology
• 出版年：2016
• 出版时间：March 2016
• 年：2016
• 卷：238
• 期：4
• 页码：584-597
• 全文大小：15440K
• ISSN：1096-9896

文摘

Well-differentiated human cancers share transcriptional programmes with the normal tissue counterparts from which they arise. These programmes broadly influence cell behaviour and function and are integral modulators of malignancy. Here, we show that the master regulator of motile ciliogenesis, FOXJ1, is highly expressed in cells along the ventricular surface of the human brain. Strong expression is present in cells of the ependyma and the choroid plexus as well as in a subset of cells residing in the subventricular zone. Expression of FOXJ1 and its transcriptional programme is maintained in many well-differentiated human tumours that arise along the ventricle, including low-grade ependymal tumours and choroid plexus papillomas. Anaplastic ependymomas as well as choroid plexus carcinomas show decreased FOXJ1 expression and its associated ciliogenesis programme genes. In ependymomas and choroid plexus tumours, reduced expression of FOXJ1 and its ciliogenesis programme are markers of poor outcome and are therefore useful biomarkers for assessing these tumours. Transitions in ciliogenesis define distinct differentiation states in ependymal and choroid plexus tumours with important implications for patient care. Copyright