Abnormal prion protein in the pituitary in sporadic and variant Creutzfeldt-Jakob disease.
By using high-sensitivity Western blotting and immunohistochemistry, pituitary glands from patients with sporadic and variant Creutzfeldt-Jakob disease (sCJD and vCJD, respectively) were analysed for the presence of the protease-resistant form of the prion protein (PrPres). PrPres was detected in a greater proportion of vCJD pituitaries than sCJD pituitaries and was localized predominantly in the neurohypophysis. PrPres was also detected in a recurrent pituitary adenoma from an sCJD patient. Immunohistochemical analysis showed sparse positive labelling, predominantly in folliculostellate cells, in vCJD and sCJD adenohypophyses. The PrPres glycosylation pattern in the vCJD neurohypophyses showed a predominance of the unglycosylated band, which differed markedly from patterns found in all other vCJD tissues. The presence of PrPres in the pituitary of CJD patients at autopsy suggests that human growth hormone-related iatrogenic CJD may have indeed resulted from infectivity in collected pituitaries rather than necessarily from contamination of pituitary pools by adjacent brain tissue.
Peden
Alexander H
AH
National Creutzfeldt-Jakob Disease Surveillance Unit (NCJDSU) and Division of Pathology, School of Molecular and Clinical Medicine, University of Edinburgh, Western General Hospital, Edinburgh, UK.
Ritchie
Diane L
DL
National Creutzfeldt-Jakob Disease Surveillance Unit (NCJDSU) and Division of Pathology, School of Molecular and Clinical Medicine, University of Edinburgh, Western General Hospital, Edinburgh, UK.
Uddin
Hafsana P
HP
National Creutzfeldt-Jakob Disease Surveillance Unit (NCJDSU) and Division of Pathology, School of Molecular and Clinical Medicine, University of Edinburgh, Western General Hospital, Edinburgh, UK.
Dean
Andrew F
AF
Addenbrooke's Hospital, Cambridge University Hospitals NHS Foundation Trust, Hills Road, Cambridge, UK.
Schiller
Kimberley A F
KAF
National Creutzfeldt-Jakob Disease Surveillance Unit (NCJDSU) and Division of Pathology, School of Molecular and Clinical Medicine, University of Edinburgh, Western General Hospital, Edinburgh, UK.
Head
Mark W
MW
National Creutzfeldt-Jakob Disease Surveillance Unit (NCJDSU) and Division of Pathology, School of Molecular and Clinical Medicine, University of Edinburgh, Western General Hospital, Edinburgh, UK.
Ironside
James W
JW
National Creutzfeldt-Jakob Disease Surveillance Unit (NCJDSU) and Division of Pathology, School of Molecular and Clinical Medicine, University of Edinburgh, Western General Hospital, Edinburgh, UK.
D015153:Blotting, Western; D007562:Creutzfeldt-Jakob Syndrome / Q000378:metabolism* / Q000473:pathology*; D006031:Glycosylation; D006801:Humans; D007150:Immunohistochemistry; D010902:Pituitary Gland / Q000737:chemistry* / Q000473:pathology; D010903:Pituitary Gland, Anterior / Q000737:chemistry / Q000473:pathology; D010904:Pituitary Gland, Posterior / Q000737:chemistry / Q000473:pathology; D011328:Prions / Q000032:analysis*
D011328:Prions