GFAP is necessary for the integrity of CNS white matter architecture and long-term maintenance of myelination

Wolfgang Liedtke, Winfried Edelmann, Phyllis L. Bieri, Fung Chow Chiu, Nicholas J. Cowan, Raju Kucherlapati, Cedric S. Raine

Research output: Contribution to journalArticle

364 Scopus citations

Abstract

To investigate the structural role of glial fibrillary acidic protein (GFAP) in vivo, mice carrying a null mutation in GFAP were generated. In 7/14 mutant animals older than 18 months of age, hydrocephalus associated with white matter loss was detected. Mutant mice displayed abnormal myelination including the presence of actively myelinating oligodendrocytes in adults, nonmyelinated axons in optic nerve, and reduced myelin thickness in spinal cord. White matter was poorly vascularized and the blood-brain harrier was structurally and functionally impaired. Astrocytic structure and function were abnormal, consisting of shortened astrocytic cell processes, decreased septation of white matter, and increased CNS extracellular space. Thus, GFAP expression is essential for normal white matter architecture and blood-brain barrier integrity, and its absence leads to late-onset CNS dysmyelination.

Original languageEnglish (US)
Pages (from-to)607-615
Number of pages9
JournalNeuron
Volume17
Issue number4
DOIs
StatePublished - Oct 1996

ASJC Scopus subject areas

  • Neuroscience(all)

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