Nearly ubiquitous tissue distribution of the scrapie agent precursor protein.
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.
Bendheim
P E
PE
Department of Molecular Biology, New York State Institute for Basic Research, Staten Island.
Brown
H R
HR
Rudelli
R D
RD
Scala
L J
LJ
Goller
N L
NL
Wen
G Y
GY
Kascsak
R J
RJ
Cashman
N R
NR
Bolton
D C
DC
D000818:Animals; D001921:Brain / Q000378:metabolism; D006224:Cricetinae; D005753:Gastric Mucosa / Q000378:metabolism; D007150:Immunohistochemistry; D008168:Lung / Q000166:cytology / Q000378:metabolism / Q000648:ultrastructure; D016253:Microscopy, Immunoelectron; D018620:PrPSc Proteins; D011328:Prions / Q000378:metabolism*; D013116:Spinal Cord / Q000166:cytology / Q000378:metabolism; D013270:Stomach / Q000166:cytology; D014018:Tissue Distribution
D018620:PrPSc Proteins; D011328:Prions