Breaches of the pial basement membrane and disappearance of the glia limitans during development underlie the cortical lamination defect in the mouse model of muscle-eye-brain disease.
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COL4A1 mutations cause ocular dysgenesis, neuronal localization defects, and myopathy in mice and Walker-Warburg syndrome in humansMutation of the BiP/GRP78 gene causes axon outgrowth and fasciculation defects in the thalamocortical connections of the mammalian forebrainGPR56-regulated granule cell adhesion is essential for rostral cerebellar development.LARGE expression augments the glycosylation of glycoproteins in addition to α-dystroglycan conferring laminin binding.Polymicrogyria: pathology, fetal origins and mechanisms.Ectopic clustering of Cajal-Retzius and subplate cells is an initial pathological feature in Pomgnt2-knockout mice, a model of dystroglycanopathy.Cortical dysplasia and skull defects in mice with a Foxc1 allele reveal the role of meningeal differentiation in regulating cortical development.Protein O-Mannosylation in the Murine Brain: Occurrence of Mono-O-Mannosyl Glycans and Identification of New SubstratesDevelopmental expression of the neuron-specific N-acetylglucosaminyltransferase Vb (GnT-Vb/IX) and identification of its in vivo glycan products in comparison with those of its paralog, GnT-VDystroglycan on radial glia end feet is required for pial basement membrane integrity and columnar organization of the developing cerebral cortex.Conditional knockout of protein O-mannosyltransferase 2 reveals tissue-specific roles of O-mannosyl glycosylation in brain development.Muscular dystrophies due to glycosylation defects.Postnatal Gene Therapy Improves Spatial Learning Despite the Presence of Neuronal Ectopia in a Model of Neuronal Migration Disorder.Abnormal development of the human cerebral cortex.Cell-autonomous and cell-to-cell signalling events in normal and altered neuronal migration.Lissencephaly with brainstem and cerebellar hypoplasia and congenital cataracts.Analysis of preplate splitting and early cortical development illuminates the biology of neurological disease.RPTPζ/phosphacan is abnormally glycosylated in a model of muscle-eye-brain disease lacking functional POMGnT1β2 and γ3 laminins are critical cortical basement membrane components: ablation of Lamb2 and Lamc3 genes disrupts cortical lamination and produces dysplasia.CXCR4 signaling regulates radial glial morphology and cell fate during embryonic spinal cord development.Evidence of early defects in Cajal-Retzius cell localization during brain development in a mouse model of dystroglycanopathy.Vitamin C Depletion in Prenatal Guinea Pigs as a Model of Lissencephaly Type II.Degree of Cajal-Retzius Cell Mislocalization Correlates with the Severity of Structural Brain Defects in Mouse Models of Dystroglycanopathy.
P2860
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P2860
Breaches of the pial basement membrane and disappearance of the glia limitans during development underlie the cortical lamination defect in the mouse model of muscle-eye-brain disease.
description
2007 nî lūn-bûn
@nan
2007年の論文
@ja
2007年学术文章
@wuu
2007年学术文章
@zh-cn
2007年学术文章
@zh-hans
2007年学术文章
@zh-my
2007年学术文章
@zh-sg
2007年學術文章
@yue
2007年學術文章
@zh
2007年學術文章
@zh-hant
name
Breaches of the pial basement ...... l of muscle-eye-brain disease.
@en
Breaches of the pial basement ...... l of muscle-eye-brain disease.
@nl
type
label
Breaches of the pial basement ...... l of muscle-eye-brain disease.
@en
Breaches of the pial basement ...... l of muscle-eye-brain disease.
@nl
prefLabel
Breaches of the pial basement ...... l of muscle-eye-brain disease.
@en
Breaches of the pial basement ...... l of muscle-eye-brain disease.
@nl
P2093
P356
P1476
Breaches of the pial basement ...... l of muscle-eye-brain disease.
@en
P2093
P2860
P304
P356
10.1002/CNE.21238
P407
P577
2007-05-01T00:00:00Z