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Karen-Marie Pedersen

Neurological disturbances, premature lethality, and central myelination deficiency in transgenic mice overexpressing the homeo domain transcription factor Oct-6

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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.
Original languageEnglish
JournalJournal of Clinical Investigation
Volume101
Issue6
Pages (from-to)1292-9
Number of pages8
ISSN0021-9738
DOIs
Publication statusPublished - 1998

    Research areas

  • Animals, Axons, Blotting, Northern, Blotting, Western, Central Nervous System, Cloning, Molecular, DNA, Gene Expression Regulation, Immunohistochemistry, Mice, Mice, Inbred C57BL, Mice, Inbred DBA, Mice, Transgenic, Microscopy, Electron, Mutagenesis, Insertional, Myelin Basic Proteins, Myelin Sheath, Nervous System Diseases, Octamer Transcription Factor-6, Polymerase Chain Reaction, RNA, Transcription Factors

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