Cellular mechanisms involved in the stenosis and obliteration of the cerebral aqueduct of hyh mutant mice developing congenital hydrocephalus

C. Wagner, L. F. Batiz, S. Rodríguez, A. J. Jiménez, P. Páez, M. Tomé, J. M. Pérez-Fígares, Esteban M. Rodríguez*

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

83 Scopus citations


Two phases may be recognized in the development of congenital hydrocephalus in the hyh mutant mouse. During embryonic life the detachment of the ventral ependyma is followed by a moderate hydrocephalus. During the first postnatal week the cerebral aqueduct becomes obliterated and a severe hydrocephalus develops. The aim of the present investigation was to elucidate the cellular phenomena occurring at the site of aqueduct obliteration and the probable participation of the subcommissural organ in this process. Electron microscopy, immunocytochemistry, and lectin histochemistry were used to investigate the aqueduct of normal and hydrocephalic hyh mice from embryonic day 14 (E-14) to postnatal day 7 (PN-7). In the normal hyh mouse, the aqueduct is an irregularly shaped cavity with 3 distinct regions (rostral, middle, and caudal) lined by various types of ependyma. In the hydrocephalic mouse, these 3 regions behave differently; the rostral end becomes stenosed, the middle third dilates, and the caudal end obliterates. The findings indicate that the following sequence of events lead to hydrocephalus: 1) denudation of the ventral ependyma (embryonic life); 2) denudation of dorsal ependyma and failure of the subcommissural organ to form Reissner fiber (first postnatal week); 3) obliteration of distal end of aqueduct; and 4) severe hydrocephalus. No evidence was obtained that NCAM is involved in the detachment of ependymal cells. The process of ependymal denudation would involve alterations of the surface sialoglycoproteins of the ependymal cells and the interaction of the latter with macrophages.

Original languageEnglish
Pages (from-to)1019-1040
Number of pages22
JournalJournal of Neuropathology and Experimental Neurology
Issue number10
StatePublished - 1 Oct 2003
Externally publishedYes


  • Aqueductal obliteration
  • Congenital hydrocephalus
  • Ependymal defect
  • Ependymal denudation
  • Glycoprotein defect
  • Macrophages
  • hyh mouse


Dive into the research topics of 'Cellular mechanisms involved in the stenosis and obliteration of the cerebral aqueduct of hyh mutant mice developing congenital hydrocephalus'. Together they form a unique fingerprint.

Cite this