Defects in tangential neuronal migration of pontine nuclei neurons in theLargemydmouse are associated with stalled migration in the ventrolateral hindbrain
| Autor: | James E. Crandall, Frances I. Smith, Peter McCaffery, Tuanlian Luo, Qiang Qu |
|---|---|
| Rok vydání: | 2006 |
| Předmět: |
PAX6 Transcription Factor
Intermediate Filaments Mice Transgenic Hindbrain In situ hybridization Biology Dystroglycans Mice Cell Movement Laminin Neural Pathways medicine Dystroglycan Animals Paired Box Transcription Factors RNA Messenger Amino Acids Eye Proteins Intermediate filament In Situ Hybridization Homeodomain Proteins Neurons Caspase 3 Reverse Transcriptase Polymerase Chain Reaction General Neuroscience Pontine nuclei Gene Expression Regulation Developmental Glycosyltransferases Embryo Mammalian Immunohistochemistry Repressor Proteins Rhombencephalon medicine.anatomical_structure Animals Newborn Bromodeoxyuridine nervous system Caspases biology.protein Basal lamina Neuroscience |
| Zdroj: | European Journal of Neuroscience. 23:2877-2886 |
| ISSN: | 1460-9568 0953-816X |
| DOI: | 10.1111/j.1460-9568.2006.04836.x |
| Popis: | The LARGE gene encodes a putative glycosyltransferase that is required for normal glycosylation of dystroglycan, and defects in LARGE can cause abnormal neuronal migration in congenital muscular dystrophy (CMD). Previous studies have focused on radial migration, which is disrupted at least in part due to breaks in the basal lamina. Through analysis of precerebellar nuclei development in the Large(myd) mouse hindbrain, we show that tangential migration of a subgroup of hindbrain neurons may also be disrupted. Within the precerebellar nuclei, the pontine nuclei (PN) are severely disrupted, whereas the inferior olive (IO), external cuneate nuclei (ECN) and lateral reticular nuclei (LRN) appear unaffected. Large and dystroglycan are widely expressed in the hindbrain, including in the pontine neurons migrating in the anterior extramural migratory stream (AES). BrdU labeling and immunohistochemical studies suggest normal numbers of neurons begin their journey towards the ventral midline in the AES in the Large(myd) mouse. However, migration stalls and PN neurons fail to reach the midline, surviving as ectopic clusters of cells located under the pial surface dorsally and laterally to where they normally would finish their migration near the ventral midline. Stalling of PN neurons at this location is also observed in other migration disorders in mice. These observations suggest that glycan-dependent dystroglycan interactions are required for PN neurons to correctly respond to signals at this important migrational checkpoint. |
| Databáze: | OpenAIRE |
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