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oai:riuma.uma.es:10630/365382026-02-03T11:14:02Zcom_10630_2254col_10630_37953

00925njm 22002777a 4500 dc Fernández-Llebrez, Pedro author Mateos-Grondona, Jesús author Pérez-Rodríguez, Juan author López-Aranda, Manuel Francisco author Estivill-Torrús, Guillermo author Llebrez Zayas, Pedro author Soriano, E. author Ramos, C. author Lallemand, Y. author Bach, A. author Robert, B. author 2004-06 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. 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 https://hdl.handle.net/10630/36538 10.1093/jnen/63.6.574 Mutación inducida animal Hidrocefalia Msx1-deficient mice fail to form prosomere 1 derivatives, subcommissural organ, and posterior commissure and develop hydrocephalus