Neurological disturbances, premature lethality, and central myelination deficiency in transgenic mice overexpressing the homeo domain transcription factor Oct-6
| Autor: | Julio E. Celis, Karen M. Pedersen, Niels A. Jensen, Mark J. West |
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| Rok vydání: | 1998 |
| Předmět: |
Central Nervous System
Genetically modified mouse Transgene Blotting Western Central nervous system Mice Transgenic Biology Polymerase Chain Reaction Mice Myelin Myelin Basic Proteins medicine Animals Cloning Molecular Transcription factor Myelin Sheath Regulation of gene expression POU domain Myelin Basic Protein DNA General Medicine Blotting Northern Immunohistochemistry Molecular biology Axons Oligodendrocyte Cell biology Mice Inbred C57BL Microscopy Electron Mutagenesis Insertional medicine.anatomical_structure Gene Expression Regulation Mice Inbred DBA Octamer Transcription Factor-6 RNA sense organs Nervous System Diseases Research Article Transcription Factors |
| Zdroj: | Jensen, N A, Pedersen, K-M, Celis, J E & West, M J 1998, ' Neurological disturbances, premature lethality, and central myelination deficiency in transgenic mice overexpressing the homeo domain transcription factor Oct-6 ', Journal of Clinical Investigation, vol. 101, no. 6, pp. 1292-9 . https://doi.org/10.1172/JCI1807 |
| ISSN: | 0021-9738 |
| DOI: | 10.1172/jci1807 |
| Popis: | Pit, Oct, Unc (POU) homeo domain transcription factors have been implicated in various developmental processes, including cell division, differentiation, specification, and survival of specific cell types. Although expression of the transcription factor Oct-6 in oligodendroglia is confined to the promyelin stage and is downregulated at the myelin stage of development, the effect of Oct-6 overexpression on oligodendrocyte development has not been established. Here we show that transgenic animals overexpressing Oct-6 at late oligodendrocyte development develop a severe neurologic syndrome characterized by action tremors, recurrent seizures, and premature death. Axons in the central nervous system of Oct-6 transgenics were hypomyelinated, hypermyelinated, or dysmyelinated, and ultrastructural analyses suggested that myelin formation was premature. The vulnerability of developing oligodendroglia to Oct-6 deregulation provides evidence that the POU factor may play a direct role in myelin disease pathogenesis in the mammalian CNS. |
| Databáze: | OpenAIRE |
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