Failure to Detect the Presence of Prions in the Uterine and Gestational Tissues from a Gravida with Creutzfeldt-Jakob Disease
| Autor: | Wen-Quan Zou, Qingzhong Kong, Ruben Vidal, Robert E. Wyza, Alan M. Golichowski, Xiangzhu Xiao, Zhiqian Dong, Karen L. Roos, Nicholas L. King, Gregory T. MacLennan, Pierluigi Gambetti, Geoff Kneale, Bernardino Ghetti, John McGeehan, Jue Yuan, Leticia Miravalle, Salvatore Spina |
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| Rok vydání: | 2009 |
| Předmět: |
Adult
Pathology medicine.medical_specialty Amniotic fluid PrPSc Proteins Biopsy Placenta animal diseases Blotting Western Short Communications Uterus Biology Creutzfeldt-Jakob Syndrome Pathology and Forensic Medicine Cerebrospinal fluid Degenerative disease Pregnancy mental disorders medicine Humans Brain Amniotic Fluid medicine.disease Virology nervous system diseases medicine.anatomical_structure Female Autopsy Endopeptidase K |
| Zdroj: | The American Journal of Pathology. 174:1602-1608 |
| ISSN: | 0002-9440 |
| Popis: | The vertical transmission of a prion disease from infected mothers to their offspring is believed to be one of the routes for the natural spread of animal prion diseases. Supporting this notion is the observation that prion infectivity occurs in the placenta of infected ewes. Furthermore, the prion protein (PrP), both in its cellular form (PrP(C)) and its pathological isoform (PrP(Sc)), has been observed at the fetal-maternal interface of scrapie-infected sheep. However, whether these features of prion infectivity also hold true for human prion diseases is currently unknown. To begin to address such an important question, we examined PrP in the uterus as well as gestational tissues, including the placenta and amniotic fluid, in a pregnant woman with sporadic Creutzfeldt-Jakob disease (CJD). Although the proteinase K (PK)-resistant prion protein, PrP27-30, was present in the brain tissues of the mother, the PrP detected in the uterus, placenta, and amniotic fluid was sensitive to PK digestion. Unlike PrP(C) in the brain and adjacent cerebrospinal fluid, the predominant PrP species in the reproductive and gestational tissues were N-terminally truncated, similar to urine PrP. Our study did not detect abnormal PrP in the reproductive and gestational tissues in this case of CJD. Nevertheless, examination by a highly sensitive bioassay is ongoing to ascertain possible prion infectivity from CJD in the amniotic fluid. |
| Databáze: | OpenAIRE |
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