A Programmed Ependymal Denudation Precedes Congenital Hydrocephalus in thehyhMutant Mouse
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چکیده
منابع مشابه
A temporo-spatial programmed ependymal denudation leads to hydrocephalus in the hyh mutant mouse
*Address correspondence to: A.J. Jiménez. Departamento de Biología Celular y Genética, Facultad de Ciencias, Universidad de Málaga, Spain. e-mail: [email protected] ABSTRACT Hyh is a lethal autosomal recessive mouse mutation located in chromosome 7. Newborn homozygous mutants presented moderate hydrocephalus, and then a severe hydrocephalus develops during the first postnatal days (PérezFígares ...
متن کاملEpendymal denudation and alterations of the subventricular zone occur in human fetuses with a moderate communicating hydrocephalus.
In mutant rodents, ependymal denudation occurs early in fetal life, preceding the onset of a communicating hydrocephalus, and is a key event in the etiology of this disease. The present investigation was designed to obtain evidence whether or not ependymal denudation occurs in 16- to 40-week-old human fetuses developing a communicating hydrocephalus (n = 8) as compared to fetuses of similar age...
متن کاملCellular mechanisms involved in the stenosis and obliteration of the cerebral aqueduct of hyh mutant mice developing congenital hydrocephalus.
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 occurr...
متن کاملDecompensated chronic congenital hydrocephalus.
A 66 year-old woman with congenital macro cephaly and developmental delay presented with aphasia and right-sided weakness. Head CT revealed severe hydrocephalus of the lateral and third ventricles with a normal fourth ventricle due to a chronic congenital process (Fig. 1A). MRI confirmed subacute embolic infarcts of the left middle cerebral artery territory (Fig. 1B) and a heparin drip was init...
متن کاملDeletion of the Parkin co-regulated gene causes defects in ependymal ciliary motility and hydrocephalus in the quakingviable mutant mouse.
The quakingviable mouse (qkv) is a spontaneous recessive mouse mutant with a deletion of approximately 1.1 Mb in the proximal region of chromosome 17. The deletion affects the expression of three genes; quaking (Qk), Parkin-coregulated gene (Pacrg) and parkin (Park2). The resulting phenotype, which includes dysmyelination of the central nervous system and male sterility, is due to reduced expre...
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ژورنال
عنوان ژورنال: Journal of Neuropathology & Experimental Neurology
سال: 2001
ISSN: 0022-3069,1554-6578
DOI: 10.1093/jnen/60.11.1105