Abstract:
: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.
journal_name
Neurologyjournal_title
Neurologyauthors
Bendheim PE,Brown HR,Rudelli RD,Scala LJ,Goller NL,Wen GY,Kascsak RJ,Cashman NR,Bolton DCdoi
10.1212/wnl.42.1.149subject
Has Abstractpub_date
1992-01-01 00:00:00pages
149-56issue
1eissn
0028-3878issn
1526-632Xjournal_volume
42pub_type
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