Patterned neuropathologic events occurring in hyh congenital hydrocephalic mutant mice

Patricia Páez, Luis Federico Bátiz, Ruth Roales-Buján, Luis Manuel Rodríguez-Pérez, Sara Rodríguez, Antonio Jesús Jiménez*, Esteban Martín Rodríguez, José Manuel Pérez-Fígares

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

55 Scopus citations


Hyh mutant mice develop long-lasting hydrocephalus and represent a good model for investigating neuropathologic events associated with hydrocephalus. The study of their brains by use of lectin binding, bromodeoxyuridine labeling, immunochemistry, and scanning electron microscopy revealed that certain events related to hydrocephalus followed a well-defined pattern. A program of neuroepithelium/ependyma denudation was initiated at embryonic day 12 and terminated at the end of the second postnatal week. After the third postnatal week the denuded areas remained permanently devoid of ependyma. In contrast, a selective group of ependymal areas resisted denudation throughout the lifespan. Ependymal denudation triggered neighboring astrocytes to proliferate. These astrocytes expressed particular glial markers and formed a superficial cell layer replacing the lost ependyma. The loss of the neuroepithelium/ependyma layer at specific regions of the ventricular walls and at specific stages of brain development would explain the fact that only certain brain structures had abnormal development. Therefore, commissural axons forming the corpus callosum and the hippocampal commissure displayed abnormalities, whereas those forming the anterior and posterior commissures did not; and the brain cortex was not homogenously affected, with the cingular and frontal cortices being the most altered regions. All of these telencephalic alterations developed at stages when hydrocephalus was not yet patent at the lateral ventricles, indicating that abnormal neural development and hydrocephalus are linked at the etiologic level, rather than the former being a consequence of the latter. All evidence collected on hydrocephalic hyh mutant mice indicates that a primary alteration in the neuroepithelium/ependyma cell lineage triggers both hydrocephalus and abnormalities in telencephalic development.

Original languageEnglish
Pages (from-to)1082-1092
Number of pages11
JournalJournal of Neuropathology and Experimental Neurology
Issue number12
StatePublished - Dec 2007
Externally publishedYes


  • Astroglial response
  • Corpus callosum agenesis
  • Cortical disorders
  • Hyh mutant
  • Inherited hydrocephalus
  • Neurodegenerative process
  • Neuroepithelium/ependyma defect


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