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J. Biol. Chem., Vol. 276, Issue 21, 18229-18234, May 25, 2001
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From the Scrapie is a naturally occurring prion
(PrP) disease causing a fatal neurodegenerative disorder in
sheep and goats. Previous studies suggest that scrapie is transmitted
naturally through exposure to the scrapie agent in wasted placentas of
infected ewes. This study determined the distribution and biochemical
properties of PrP cellular (PrP-C) and the distribution of PrP
scrapie (PrP-Sc) in reproductive, placental, and selected
fetal tissues and fetal fluids in sheep. Glycosylated, N-terminally
truncated, proteinase K-sensitive PrP-C with apparent molecular
masses of 23-37 kDa was present in reproductive, placental, and
fetal tissues and fetal fluids. PrP-C was low or undetectable in
intercotyledonary chorioallantois, amnion, urachus, amniotic fluid, and
fetal urine. In pregnant ewes, cotyledonary chorioallantois, allantoic
fluid, and caruncular endometrium contained higher levels of PrP-C than did intercaruncular endometrium, myometrium, oviduct, ovary, fetal bladder, or fetal kidney. Caruncular endometrial PrP-C was up-regulated during pregnancy. Despite the wide distribution of PrP-C in
reproductive, placental, and selected fetal tissues and fetal fluid,
PrP-Sc was detected only in caruncular endometrium and cotyledonary
chorioallantois of pregnant scrapie-infected ewes. The embryo/fetus may
not be exposed to scrapie in utero because it is separated
physically from PrP-positive allantois and chorioallantois by
PrP-negative amnion.
PrP-C and PrP-Sc at the Fetal-Maternal Interface*
§,¶,
, and
Animal Disease Research Unit, Agricultural
Research Service, United States Department of Agriculture, Pullman,
Washington 99164, the ¶ Department of Veterinary Microbiology and
Pathology, College of Veterinary Medicine, Washington State University,
Pullman, Washington 99164, and the Institute of Pathology, Case
Western Reserve University, Cleveland, Ohio 44106
*
This work was supported by grants from the Agricultural
Research Services, United States Department of Agriculture Grant CWU 5348-32000-015-00D.The costs of publication of this
article were defrayed in part by the
payment of page charges. The article
must therefore be hereby marked
"advertisement" in
accordance with 18 U.S.C. Section
1734 solely to indicate this fact.
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