title: Decreased FOXJ1 expression and its ciliogenesis programme in aggressive ependymoma and choroid plexus tumours.
abstract: 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 © 2015 Pathological Society of Great Britain and Ireland. Published by John Wiley & Sons, Ltd.
authors.affiliation: Department of Pathology, Division of Neuropathology, Brigham and Women's Hospital, Harvard Medical School, Boston, MA, USA.|Department of Pathology, King Fahad Medical City, Riyadh, Saudi Arabia.|King Abdulaziz City for Science and Technology, Riyadh, Saudi Arabia.|Department of Pathology, Boston Children's Hospital, Harvard Medical School, Boston, MA, USA.
authors.forename: Malak S
authors.initials: MS
authors.lastname: Abedalthagafi
authors.affiliation: Department of Pathology, Division of Neuropathology, Brigham and Women's Hospital, Harvard Medical School, Boston, MA, USA.
authors.forename: Michael P
authors.initials: MP
authors.lastname: Wu
authors.affiliation: Department of Pathology, Division of Neuropathology, Brigham and Women's Hospital, Harvard Medical School, Boston, MA, USA.
authors.forename: Parker H
authors.initials: PH
authors.lastname: Merrill
authors.affiliation: Department of Pathology, Division of Neuropathology, Brigham and Women's Hospital, Harvard Medical School, Boston, MA, USA.
authors.forename: Ziming
authors.initials: Z
authors.lastname: Du
authors.affiliation: Department of Pathology, Division of Neuropathology, Brigham and Women's Hospital, Harvard Medical School, Boston, MA, USA.
authors.forename: Terri
authors.initials: T
authors.lastname: Woo
authors.affiliation: Department of Pathology, Boston Children's Hospital, Harvard Medical School, Boston, MA, USA.
authors.forename: Shu-Hsien
authors.initials: SH
authors.lastname: Sheu
authors.affiliation: Department of Medicine, Brigham and Women's Hospital, Harvard Medical School, Boston, MA, USA.
authors.forename: Shelley
authors.initials: S
authors.lastname: Hurwitz
authors.affiliation: Department of Pathology, Division of Neuropathology, Brigham and Women's Hospital, Harvard Medical School, Boston, MA, USA.|Department of Pathology, Boston Children's Hospital, Harvard Medical School, Boston, MA, USA.|Department of Medical Oncology, Dana-Farber Cancer Institute, Boston, MA, USA.
authors.forename: Keith L
authors.initials: KL
authors.lastname: Ligon
authors.affiliation: Department of Pathology, Division of Neuropathology, Brigham and Women's Hospital, Harvard Medical School, Boston, MA, USA.|Department of Pathology, Boston Children's Hospital, Harvard Medical School, Boston, MA, USA.|Department of Cancer Biology, Dana-Farber Cancer Institute, Boston, MA, USA.
authors.forename: Sandro
authors.initials: S
authors.lastname: Santagata
mesh_terms: D001932:Brain Neoplasms / Q000235:genetics / Q000378:metabolism* / Q000473:pathology*; D002454:Cell Differentiation / Q000502:physiology; D016545:Choroid Plexus Neoplasms / Q000235:genetics / Q000378:metabolism* / Q000473:pathology*; D004805:Ependyma / Q000378:metabolism; D004806:Ependymoma / Q000235:genetics / Q000378:metabolism*; D051858:Forkhead Transcription Factors / Q000235:genetics / Q000378:metabolism*; D006801:Humans
chemical_list: C106349:FOXJ1 protein, human; D051858:Forkhead Transcription Factors
keywords: FOXJ1; choroid plexus; cilia; ciliogenesis; dedifferentiation; ependymoma; stem cells; subventricular zone