Abstract
Background A population (30%) of hydrocephalic hyh mutant mice develop a slowly progressive hydrocephalus and survive for periods ranging between 2 months and 2 years. Certain characteristics of these mice, such as time of onset of hydrocephalus, type of abnormality of CSF dynamics, clinical evolution and survival/death rate, resemble several types of human congenital hydrocephalus. They represent an exceptional animal model to investigate neuropathological and physiopathological aspects of a brain adapting to a virtually life-lasting hydrocephalus
Highlights
Materials and methods (i) The clinical evolution of more than 3,000 hyh mice,(ii) certain cellular and molecular aspects involved in the pathogenesis of hydrocephalus of several hundreds of embryos and postnatal hyh specimens and, (iii) the neuropathology of more than one hundred hydrocephalic mice with arrested hydrocephalus have been investigated with a large series of techniques
Loss: Neuroepithelium/ependyma denudation is a severe loss, since it leads to (i) Sylvius aqueduct obliteration and severe hydrocephalus; (ii) abnormal development of certain populations of cerebral neurons resulting in a permanent neurological impairment
Gain: (i) A subpopulation of astrocytes responds to denudation by repairing the denuded areas forming an ependymal-like new barrier. (ii) Once severe hydrocephalus has been turned on, two ependymal populations located in the aqueduct and third ventricle start to proliferate allowing the large expansion of these cavities
Summary
Long-lasting hydrocephalus in hyh mutant mice: gain and loss of a brain surviving hydrocephalus. Esteban M Rodríguez*1, Patricia Páez, Federico Bátiz, Carolina Wagner, Karin Vío, Antonio J Jiménez, Sara Rodríguez, Ruth Roales-Buján, Luis M Rodrígez-Pérez and José-Manuel Pérez-Fígares. Address: 1Instituto de Histología y Patología, Universidad Austral de Chile, Valdivia, Chile and 2Departamento de Biología Celular, Facultad de Ciencias, Universidad de Málaga, Málaga, España, Instituto de Histología y Patología, Universidad Austral de Chile, Valdivia, Chile. 50th Annual Meeting of the Society for Research into Hydrocephalus and Spina Bifida Meeting abstracts – A single PDF containing all abstracts in this supplement is available here.
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