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Science 4 July 1997:
Vol. 277. no. 5322, pp. 94 - 98
DOI: 10.1126/science.277.5322.94
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Reports

Evolution of a Strain of CJD That Induces BSE-Like Plaques

Laura Manuelidis, * William Fritch, You-Gen Xi

Bovine spongiform encephalopathy (BSE) has become a public health issue because a recently evolved BSE agent has infected people, yielding an unusual form of Creutzfeld-Jakob disease (CJD). A new CJD agent that provokes similar amyloid plaques and cerebellar pathology was serially propagated. First-passage rats showed obvious clinical signs and activated microglia but had negligible PrP-res (the more protease-resistant form of host PrP) or cerebellar lesions. Microglia and astrocytes may participate in strain selection because the agent evolved, stabilized, and reproducibly provoked BSE-like disease in subsequent passages. Early vacuolar change involving activated microglia and astrocytes preceded significant PrP-res accumulation by more than 50 days. These studies reveal several inflammatory host reactions to an exogenous agent.

Section of Neuropathology, Yale Medical School, 310 Cedar Street, New Haven, CT 06510, USA.
*   To whom correspondence should be addressed. E-mail: laura.manuelidis{at}yale.edu

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