Nature Medicine
7, 361 - 364 (2001)
doi:10.1038/85515
A novel erythroid-specific marker of transmissible spongiform encephalopathiesGino Miele1, Jean Manson2
& Michael Clinton11
Division of Gene Expression and Development, Roslin Institute, Roslin, Midlothian, Scotland, UK
2
BBSRC Institute For Animal Health Neuropathogenesis Unit, Edinburgh, Scotland, UK
Correspondence should be addressed to Michael Clinton michael.clinton@bbsrc.ac.ukTransmissible 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 humans1,
2. 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 death1,
2. 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.
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