Neuroependymal Denudation is in Progress in Full‐term Human Foetal Spina Bifida Aperta
- 27 January 2011
- journal article
- Published by Wiley in Brain Pathology
- Vol. 21 (2), 163-179
- https://doi.org/10.1111/j.1750-3639.2010.00432.x
Abstract
In human spina bifida aperta (SBA), cerebral pathogenesis [hydrocephalus, Sylvius aqueduct (SA) stenosis and heterotopias] is poorly understood. In animal models, loss of ventricular lining (ependymal denudation) causes SA stenosis and hydrocephalus. We aimed to investigate whether ependymal denudation also takes place in human foetal SBA. Considering that ependymal denudation would be related to alterations in junction proteins, sections through SA of five SBA and six control foetuses (gestational ages ranged between 37 and 40 weeks) were immunostained for markers of ependyma (caveolin 1, βIV‐tubulin, S100), junction proteins (N‐cadherin, connexin‐43, neural cell adhesion molecule (NCAM), blood vessels (Glut‐1) and astrocytes [glial fibrillary acidic protein (GFAP)]. In control foetuses, ependymal denudation was absent. In SBA foetuses different stages of ependymal denudation were observed: (i) intact ependyma/neuroepithelium; (ii) imminent ependymal denudation (with abnormal subcellular location of junction proteins); (iii) ependymal denudation (with protrusion of neuropile into SA, formation of rosettes and macrophage invasion); (iv) astroglial reaction. It is suggested that abnormalities in the formation of gap and adherent junctions result in defective ependymal coupling, desynchronized ciliary beating and ependymal denudation, leading to hydrocephalus. The presence of various stages of ependymal denudation within the same full‐term SBA foetuses suggests continuation of the process after birth.Keywords
This publication has 45 references indexed in Scilit:
- Neuropsychological assessment of attention in children with spina bifidaFluids and Barriers of the CNS, 2009
- Connexins: a myriad of functions extending beyond assembly of gap junction channelsCell Communication and Signaling, 2009
- Pathogenesis of cerebral malformations in human fetuses with meningomyeloceleFluids and Barriers of the CNS, 2008
- Loss of Cell Adhesion Causes Hydrocephalus in Nonmuscle Myosin II-B–ablated and Mutated MiceMolecular Biology of the Cell, 2007
- N-cadherin mediates cortical organization in the mouse brainDevelopmental Biology, 2006
- Structural abnormalities develop in the brain after ablation of the gene encoding nonmuscle myosin II‐B heavy chainJournal of Comparative Neurology, 2001
- Role of L1 in Neural Development: What the Knockouts Tell UsMolecular and Cellular Neuroscience, 1998
- Intrinsic Polarity of Mammalian Neuroepithelial CellsMolecular and Cellular Neuroscience, 1998
- Mutations in the cell adhesion molecule LI cause mental retardationTrends in Neurosciences, 1995
- Spatial and temporal expression pattern of N-cadherin cell adhesion molecules correlated with morphogenetic processes of chicken embryosDevelopmental Biology, 1987