NR AIIN
AU Miele,G.; Manson,J.; Clinton,M.
TI A novel erythroid-specific marker of transmissible spongiform encephalopathies
QU Nature Medicine 2001 Mar; 7(3): 361-4
KI Nat Med. 2001 Jun;7(6):641-2. PMID: 11385477 Nat Med. 2001 Mar;7(3):289-90. PMID: 11231622
PT journal article
AB Transmissible spongiform encephalopathies (TSE) are a group of invariably fatal neurodegenerative diseases and include scrapie in sheep, bovine spongiform encephalopathy (BSE) in cattle, chronic wasting disease in deer and elk, and Kuru disease, Creutzfeldt-Jakob disease (CJD) and variant CJD in humans. The pathological effects of disease occur predominantly in the CNS (central nervous system), where common hallmarks include vacuolation, gliosis, accumulation of a protease-resistant, abnormally folded isoform of the prion protein (PrPsc) and neuronal cell death. Lack of understanding of the molecular mechanisms underlying disease pathogenesis, particularly in non-CNS tissues, means that there are currently no effective strategies for early diagnosis or therapeutic intervention of TSEs. Here we report the first identification of a molecular marker that is easily detectable in readily accessible tissues. We demonstrate that a dramatic decrease in expression of a transcript specific to erythroid lineage cells is a common feature of TSEs. Our findings indicate a previously unrecognized role for involvement of the erythroid lineage in the etiology of TSE pathogenesis and should provide a new focus for research into diagnostic and therapeutic strategies.
MH Activins; Animal; *Biological Markers; Cell Line; Human; Inhibins/genetics; Mice; Mice, Inbred Strains; Molecular Sequence Data; Prion Diseases/*diagnosis/genetics/metabolism; Prions/genetics/metabolism; Support, Non-U.S. Gov't
AD Division of Gene Expression and Development, Roslin Institute, Roslin, Midlothian, Scotland, UK
SP englisch
PO USA