Analysis of prion protein expression on human cutaneous dendritic cells

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Prions are infectious particles which forms abnormal isoforms of prion protein (acrapie-associated PrP) of the ubiquitous prion-related protein (PrPc). Various tissues such as spleen, lung and uterus have been reported to express PrPc. We investigated whether Langerhans cells, epidermal dendritic cells that stimulate T cells in the afferent lymph nodes expressed PrPc. We succeeded to purify LC from the skin over 95% purity and identified that LC expressed strong intensity for PrPc on cell surface membrane. In vitro expreriment showed that PrPc expression on LC was enhanced by additional stimulation of IL-1 and TNF-α : Furthermore, we next purified dendritic epidermal T cells (DETC) and found that these T cells also expressed PrPc. The experiment using PrP knockout mice showed that the PrPc expression on LC and DETC was totally diminished and that no cytokines including IL-1 and TNF-α did not modulate PrPc expression on these cells. These data revealed the dynamic change of PrPc expression of epidermal densritic cells. Our data will have some role in the understanding of the pathogenesis of PrP-related diseases and to help the future thera eutic a roaches to treat PrP-relat diseases.