Predominant localization of the LIS family of gene products to Cajal-Retzius cells and ventricular neuroepithelium in the developing human cortex.
Mutations that perturb neuronal migration provide important biological clues that can lead to an understanding of the role of specific cells and molecules in the formation of the cortex. The human neuronal migration disorder, Miller-Dieker Lissencephaly, results from a hemideletion of LIS-1, which encodes a subunit of a brain platelet-activating factor acetylhydrolase. The cellular localization of the LIS-1 gene product in human fetal brain and its normal role in neuronal migration have yet to be determined. LIS-1 belongs to a family of genes that have identical coding sequences (LIS-1 [chromosome 17] and LIS-2 [chromosome 2]). In the brain, LIS-1 is the more abundant gene as determined by Northern blot analysis. Using antibodies raised against 2 epitopes of the LIS-1/LIS-2 protein sequence, we have localized the LIS family of gene products in the developing human brain to the Cajal-Retzius cells, some subplate neurons, thalamic neurons, the ventricular neuroepithelium, and at later gestational ages, to the ependyma. Therefore, LIS-1 bears some resemblance to reelin, the gene product involved in the cortical mouse mutant reeler, in that Cajal-Retzius cells demonstrate immunolocalization. However, unlike reelin, LIS proteins are expressed not only in the Cajal Retzius cells, but also in the ventricular neuroepithelium, suggesting a potential role for this structure in neuronal migration.
Clark
G D
GD
Department of Pediatrics, Baylor College of Medicine, Houston, Texas 77030, USA.
Mizuguchi
M
M
Antalffy
B
B
Barnes
J
J
Armstrong
D
D
D043203:1-Alkyl-2-acetylglycerophosphocholine Esterase; D002540:Cerebral Cortex / Q000166:cytology / Q000196:embryology* / Q000378:metabolism*; D002552:Cerebral Ventricles / Q000166:cytology / Q000196:embryology* / Q000378:metabolism*; D004622:Embryo, Mammalian / Q000166:cytology / Q000378:metabolism / Q000502:physiology; D005314:Embryonic and Fetal Development; D004847:Epithelial Cells; D004848:Epithelium / Q000196:embryology / Q000378:metabolism; D005865:Gestational Age; D006801:Humans; D008869:Microtubule-Associated Proteins; D009474:Neurons / Q000378:metabolism; D011506:Proteins / Q000378:metabolism*; D000091042:Reelin Protein; D014018:Tissue Distribution
D008869:Microtubule-Associated Proteins; D011506:Proteins; D000091042:Reelin Protein; D043203:1-Alkyl-2-acetylglycerophosphocholine Esterase; C084102:PAFAH1B1 protein, human; C513190:Pafah1b1 protein, mouse; C000717127:RELN protein, human; C000717128:Reln protein, mouse