RT Journal Article
T1 Msx1-deficient mice fail to form prosomere 1 derivatives, subcommissural organ, and posterior commissure and develop hydrocephalus
A1 Fernández-Llebrez, Pedro
A1 Mateos-Grondona, Jesús
A1 Pérez-Rodríguez, Juan
A1 López-Aranda, Manuel Francisco
A1 Estivill-Torrús, Guillermo
A1 Llebrez Zayas, Pedro
A1 Soriano, E.
A1 Ramos, C.
A1 Lallemand, Y.
A1 Bach, A.
A1 Robert, B.
K1 Mutación inducida animal
K1 Hidrocefalia
AB Msx1 is a regulatory gene involved in epithelio-mesenchymal interactions in limb formation and organogenesis. In the embryonic CNS, the Msx1 gene is expressed along the dorsal midline. Msx1 mutant mice have been obtained by insertion of the nlacZ gene in the Msx1 homeodomain. The most important features of homozygous mutants that we observed were the absence or malformation of the posterior commissure (PC) and of the subcommissural organ (SCO), the collapse of the cerebral aqueduct, and the development of hydrocephalus. Heterozygous mutants developed abnormal PC and reduced SCO, as revealed by specific antibodies against SCO secretory glycoproteins. About one third of the heterozygous mutants also showed hydrocephalus. Other defects displayed by homozygous mutants were ependymal denudation, subventricular cavitations and edema, and underdevelopment of the pineal gland and subfornical organ. Some homozygous mutants developed both SCO and PC, probably as a consequence of genetic redundancy with Msx2. However, these mutants did not show SCO-immunoreactive glycoproteins and displayed obstructive hydrocephalus. This suggests that Msx1 is necessary for the synthesis of SCO glycoproteins, which would then be required for the maintenance of an open aqueduct.
PB Oxford University Press
YR 2004
FD 2004-06
LK https://hdl.handle.net/10630/36538
UL https://hdl.handle.net/10630/36538
LA eng
NO FP. Fernández-Llebrez, J. M. Grondona, J. Pérez, M. F. López-Aranda, G. Estivill-Torrús, P. F. Llebrez-Zayas, E. Soriano, C. Ramos, Y. Lallemand, A. Bach, B. Robert, Msx1-Deficient Mice Fail to Form Prosomere 1 Derivatives, Subcommissural Organ, and Posterior Commissure and Develop Hydrocephalus, Journal of Neuropathology & Experimental Neurology, Volume 63, Issue 6, June 2004, Pages 574–586, https://doi.org/10.1093/jnen/63.6.574
NO Supported by DGICYT (BFI 2000-1360; BFI 2003-03348, Spain), FIS (01-0948; PIO2-1517, Spain), Red CIEN (Fundacio´n Hospital Carlos Haya; ISCIII, Spain) and by the Institut Pasteur, the CNRS, and grants from the Association pour la Recherche sur le Cancer and the Association Franc ¸aise contre les Myopathies.
DS RIUMA. Repositorio Institucional de la Universidad de Málaga
RD 20 ene 2026