Cited by 256Prion Diseases and Protein MisfoldingAlzheimer's disease research and treatmentsSkin and Cellular Biology Research

Mice devoid of the glial fibrillary acidic protein develop normally and are susceptible to scrapie prions

Open access
Gomi, Hiroshi, Yokoyama, Takashi, Fujimoto, Kazushi, Ikeda, Toshio, Katoh, Akira, Itoh, Takeshi, Itohara, Shigeyoshi·1995·Neuron

Abstract

Glial fibrillary acidic protein (GFAP) is an intermediate filament protein specifically expressed in astrocytes in the CNS. To examine the function of GFAP in vivo, the Gfap gene was disrupted by gene targeting in embryonic stem cells. Mice homozygous for the mutation were completely devoid of GFAP but exhibited normal development and showed no obvious anatomical abnormalities in the CNS. When inoculated with infectious scrapie prions, the mutant mice exhibited neuropathological changes typical of prion diseases. Infectious prions accumulated in brains of the mutant mice to a degree similar to that in control littermates. These results suggest that GFAP is not essential for the morphogenesis of the CNS or for astrocytic responses against neuronal injury. The results argue against the hypothesis that GFAP plays a crucial role in the pathogenesis of prion diseases.

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Crossref · 10.1016/0896-6273(95)90238-4

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67 of 67 references verified0 citation errors · 0 not checkable

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  • Absence of GFAP and/or Vim in mice has no major effect under physiological conditions.

    “Absence of GFAP and/or Vim in mice has no major effect under physiological conditions (17–20), reflecting the role of intermediate filament (nanofilament) system as a signaling platform in situations connected with cellular stress (21).”

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Authors
Gomi, H., Yokoyama, T., Fujimoto, K., Ikeda, T., Katoh, A., Itoh, T., & Itohara, S.
Affiliations
  • Kyoto University (JP)
  • National Institute of Animal Health (JP)
Venue
Neuron
Publisher
Elsevier BV
Published
1995-01
Volume
14
Issue
1
Pages
29-41
ISSN
0896-6273 (ISSN-L)
OpenAlex
W2059028264
Cited by
256
References
67
Citations by year
2026: 1 · 2025: 2 · 2024: 3 · 2023: 1 · 2022: 4 · 2021: 6 · 2020: 5 · 2019: 1 · 2017: 8 · 2016: 4 · 2015: 6 · 2014: 4 · 2013: 6 · 2012: 6
FWCI
7.99
Type
journal article
Language
en
Open access
Locations
  • Neuron (publishedVersion)
  • PubMed (publishedVersion)
Funders
  • Japan Science and Technology Agency
  • Ministry of Education, Culture, Sports, Science and Technology
Topics
  • Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Prion Diseases and Protein Misfolding
  • Health Sciences › Medicine › Physiology › Alzheimer's disease research and treatments
  • Life Sciences › Biochemistry, Genetics and Molecular Biology › Cell Biology › Skin and Cellular Biology Research
Keywords
glial fibrillary acidic protein, prion disease, astrocytes, intermediate filaments, gene targeting, central nervous system development, prion accumulation, neuropathology, prion pathogenesis
MeSH terms
Animals, Astrocytes, beta-Galactosidase, Brain, Brain Chemistry, Female, Glial Fibrillary Acidic Protein, Male, Mice, Inbred C57BL, Mice, Mutant Strains, Prions, RNA, Messenger, Scrapie, Spinal Cord, Vimentin, Mutagenesis, Gene Targeting, Mice

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