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The Journal of Neuroscience, June 30, 2004, 24(26):5974-5981; doi:10.1523/JNEUROSCI.0122-04.2004

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Neurobiology of Disease
Intrinsic Resistance of Oligodendrocytes to Prion Infection

Marco Prinz,^1 * Fabio Montrasio,^1 * Hisako Furukawa,¹ Marjan E. van der Haar,² Petra Schwarz,¹ Thomas Rülicke,³ Olivier T. Giger,¹ Karl-Georg Häusler,¹ Daniel Perez,¹ Markus Glatzel,¹ and Adriano Aguzzi¹

¹Institute of Neuropathology, University Hospital of Zürich, CH-8091 Zürich, Switzerland, ²Brain Research Institute, Department of Neuromorphology, University of Zürich, CH-8057 Zürich, Switzerland, and ³Institute of Laboratory Animal Science, University of Zürich, CH-8091 Zürich, Switzerland

Within the CNS, the normal form of cellular prion protein (PrP^C) is expressed on neurons, oligodendrocytes, and astrocytes. The contribution of these cell types to prion replication and pathogenesis is unclear. To assess the role of oligodendrocytes, we expressed PrP^C under the control of the myelin basic protein (MBP) promoter in mice lacking endogenous PrP^C. PrP^C was detected in oligodendrocytes and Schwann cells but not in neurons and astrocytes. MBP-PrP mice never developed scrapie after intracerebral, intraperitoneal, or intraocular challenge with scrapie prions. Transgenic brains did not contain protease-resistant prion protein and did not transmit scrapie when inoculated into PrP^C-overexpressing indicator mice. To investigate whether prion spread within the CNS depends on oligodendrocytic PrP^C, we implanted PrP^C-overexpressing neuroectodermal grafts into MBP-PrP brains. After intraocular prion inoculation, none of the grafts showed spongiform encephalopathy or prion infectivity. Hence oligodendrocytes do not support cell-autonomous prion replication, establishment of subclinical disease, and neural spread of prions. Prion resistance sets oligodendrocytes aside from both neurons and astrocytes.

Key words: prion; PrP^Sc; oligodendrocytes; myelin basic protein; transgenic mice; scrapie

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Received Jan 13, 2004; revised April 21, 2004; accepted May 14, 2004.

This article has been cited by other articles:

				J. I. Ayers, A. E. Kincaid, and J. C. Bartz Prion Strain Targeting Independent of Strain-Specific Neuronal Tropism J. Virol., January 1, 2009; 83(1): 81 - 87. [Abstract] [Full Text] [PDF]

				N. Genoud, D. Ott, N. Braun, M. Prinz, P. Schwarz, U. Suter, D. Trono, and A. Aguzzi Antiprion Prophylaxis by Gene Transfer of a Soluble Prion Antagonist Am. J. Pathol., May 1, 2008; 172(5): 1287 - 1296. [Abstract] [Full Text] [PDF]

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