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From the Departments of Molecular Biology (Dr. Bendheim, Mr. Scala, and Dr. Bolton), Pathological Neurobiology (Mrs. Brown, Ms. Goller, and Dr. Wen), Neuropathology (Dr. Rudelli), and Virology (Dr. Kascsak), New York State Institute for Basic Research, Staten Island, NY; the Department of Neurology (Dr. Bendheim), State University of New York Health Science Center, Brooklyn, NY; and the Department of Neurology (Dr. Cashman), Montreal Neurological Institute, Montreal, PQ, Canada.
The "modified host protein" model of scrapie proposes that the transmissible agent is composed of the degradation-resistant protein, Sp33–37, and that clinical and pathologic signs result from neurotoxic accumulations of this protein. Sp33–37 is an abnormal, amyloidogenic isoform of the normally occurring cellular protein Cp33–37. This study investigated the tissue distribution of Cp33–37 in hamster. In brain, Cp33–37 was most concentrated in the hippocampal formation. Immunohistochemical studies localized Cp33–37 to neurons and surrounding neuropil in hippocampus; septal, caudate, and thalamic nuclei; dorsal root ganglia cells; and large-diameter dorsal root axons. In non-neuronal hamster tissues, Cp33–37 was detected in circulating leukocytes, heart, skeletal muscle, lung, intestinal tract, spleen, testis, ovary, and some other organs. The presence of Cp33–37 in extracerebral tissues indicates that its function is not unique to brain. These results indicate that the molecular substrate for the production of Sp33–37, the scrapie agent, and scrapie amyloid is present in a variety of cerebral and extracerebral sites.
Address correspondence and reprint requests to Dr. P.E. Bendheim, Department of Neurobiology, The Weizmann Institute of Science, Rehovot 76100, Israel.
Supported by NIH grants NS24720 (P.E.B.) and NS21349 (R.J.K.), and by the New York State Office of Mental Retardation and Developmental Disabilities.
Received March 15, 1991. Accepted for publication in final form June 18, 1991.
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