Ultrastructural pathology of Creutzfeldt-Jakob disease and fatal familial insomnia.

Abstract

In this study we examined well fixed brain biopsies of 9 sporadic CJD, 9 iatrogenic CJD cases, 2 hereditary CJD (Gly200Lys and Val203Iso), a case of vCJD and a case of fatal familial insomnia (FFI) from a new French family collected over the years at the Service de Neuropathologie, Hopital Neurologique, Lyon, France. Tubulovesicular structures (TVS) were found in every case including FFI and nvCJD. Thus, TVS, as we suggested previously are important pathologic structure which significance should be more extensively studied.

• Authors: P P Liberski, P Giraud, N Kopp
• Journal: Folia neuropathologica (Folia Neuropathol) Vol. 38 Issue 4 Pg. 171-3 ( 2000) ISSN: 1641-4640 [Print] Poland
• PMID: 11693721 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't)

Chemical References

• Amyloid
• Codon
• PRNP protein, human
• Prion Proteins
• Prions
• Protein Precursors
• Human Growth Hormone

Topics

• Amino Acid Substitution
• Amyloid (analysis, genetics)
• Biopsy
• Cerebral Cortex (chemistry, ultrastructure)
• Codon (genetics)
• Creutzfeldt-Jakob Syndrome (pathology, transmission)
• France
• Human Growth Hormone (adverse effects)
• Humans
• Iatrogenic Disease
• Microscopy, Electron
• Point Mutation
• Prion Diseases (genetics, pathology)
• Prion Proteins
• Prions
• Protein Precursors (analysis, genetics)
• Vacuoles (ultrastructure)