Congenital Hydrocephalus and Abnormal Subcommissural Organ Development in Sox3 Transgenic Mice
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منابع مشابه
Congenital Hydrocephalus and Abnormal Subcommissural Organ Development in Sox3 Transgenic Mice
Congenital hydrocephalus (CH) is a life-threatening medical condition in which excessive accumulation of CSF leads to ventricular expansion and increased intracranial pressure. Stenosis (blockage) of the Sylvian aqueduct (Aq; the narrow passageway that connects the third and fourth ventricles) is a common form of CH in humans, although the genetic basis of this condition is unknown. Mouse model...
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Huntingtin (htt) is a 350 kDa protein of unknown function, with no homologies with other known proteins. Expansion of a polyglutamine stretch at the N-terminus of htt causes Huntington's disease (HD), a dominant neurodegenerative disorder. Although it is generally accepted that HD is caused primarily by a gain-of-function mechanism, recent studies suggest that loss-of-function may also be part ...
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the foregut endoderm and the defective expansion of the midand hindgut at early somite stages. However, there remains unclear whether or not Sox17 is required for the subsequent development, maturation and maintenance of the endoderm derivatives such as livers and pancreas due to the early embryonic lethal of Sox17-null mutant embryos before 10.0 dpc. Recently, we noticed the neonatal lethality...
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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 com...
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thesis, absorption or flow of the Cerebral Spinal Fluid (CSF) and is a common developmental disability in humans. Although CH has a significant genetic component in mice and humans, it is poorly understood at the molecular level. The Subcommissural Organ (SCO), a gland residing at the dorsal opening of the Sylvian aqueduct, has been implicated in CSF flow maintenance. We have established BAC tr...
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ژورنال
عنوان ژورنال: PLoS ONE
سال: 2012
ISSN: 1932-6203
DOI: 10.1371/journal.pone.0029041