National Institute of Animal Health (NIAH)

Topics in Animal Health Research 2009

26. Intraspecies transmission of atypical bovine spongiform encephalopathy detected in Japan

Japanese

  On the basis of the biological, neuropathological, and biochemical characteristics of BSE cases, it is believed that the bovine spongiform encephalopathy (BSE) agent is a single strain (classical BSE). However, since 2003, different neuropathological and molecular phenotypes of BSE (atypical BSEs) have been reported from various countries. At present, atypical BSEs are characterized by a higher or lower molecular mass of the unglycosylated form of the disease-associated prion protein than that of classical BSE and referred to as H-type or L-type BSE, respectively. In Japan, 2 cases of atypical BSE have been identified; the second case was identified in aged beef cattle (BSE/JP24). To characterize the phenotypes of the BSE/JP24 prion propagated in its natural host, a transmission study with cattle was performed. We demonstrated the successful transmission of the BSE/JP24 prion to cattle. On the basis of the incubation period, neuropathological hallmarks, and molecular properties of the abnormal host prion protein, the characteristics of BSE/JP24 prion were apparently distinguishable from that of the classical BSE prion, and they closely resembled those of the L-type BSE found in Italy.
(Research Team for Prion Diseases, TEL +81-29-838-7708)

Reference:

Fukuda, S., Iwamaru, Y. et al. (2009) Microbiol. Immunol. 53:704-707.

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