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Ependymal cells: biology and pathology

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Abstract

The literature was reviewed to summarize the current understanding of the role of ciliated ependymal cells in the mammalian brain. Previous reviews were summarized. Publications from the past 10 years highlight interactions between ependymal cells and the subventricular zone and the possible role of restricted ependymal populations in neurogenesis. Ependymal cells provide trophic support and possibly metabolic support for progenitor cells. Channel proteins such as aquaporins may be important for determining water fluxes at the ventricle wall. The junctional and anchoring proteins are now fairly well understood, as are proteins related to cilia function. Defects in ependymal adhesion and cilia function can cause hydrocephalus through several different mechanisms, one possibility being loss of patency of the cerebral aqueduct. Ependymal cells are susceptible to infection by a wide range of common viruses; while they may act as a line of first defense, they eventually succumb to repeated attacks in long-lived organisms. Ciliated ependymal cells are almost certainly important during brain development. However, the widespread absence of ependymal cells from the adult human lateral ventricles suggests that they may have only regionally restricted value in the mature brain of large size.

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Acknowledgments

The author holds the Canada Research Chair in Developmental Neuropathology. The author thanks Dr. K. Kosaki (Keio University School of Medicine, Tokyo, Japan) for providing slides from a fetus with hydrocephalus and cilia disease.

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Correspondence to Marc R. Del Bigio.

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Del Bigio, M.R. Ependymal cells: biology and pathology. Acta Neuropathol 119, 55–73 (2010). https://doi.org/10.1007/s00401-009-0624-y

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