| First Author | Miele G | Year | 2001 |
| Journal | Nat Med | Volume | 7 |
| Issue | 3 | Pages | 361-4 |
| PubMed ID | 11231637 | Mgi Jnum | J:73795 |
| Mgi Id | MGI:2156813 | Doi | 10.1038/85515 |
| Citation | Miele G, et al. (2001) A novel erythroid-specific marker of transmissible spongiform encephalopathies. Nat Med 7(3):361-4 |
| abstractText | 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. |
