Abstract
Virus-like particles are commonly found in highly infectious scrapie brain fractions and cell lines, suggesting a viral source of prion diseases. However, new evidence indicates that these particles are likely the result of mucopolysaccharidosis; a lysosomal storage disease where similar particles are observed, along with neuronal degeneration. Heparan sulfate proteoglycans (HSPGs) have long been implicated in prion diseases including demonstrated impairment of glycosaminoglycan metabolism. In this work, unusual levels of glypican-1 and heparan sulfate in cell cultures treated with prion protein antibodies are disclosed, further evidence for a lysosomal storage disorder and suggestive of a HSPG origin for prion diseases.
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