Neuronal Ndst1 depletion accelerates prion protein clearance and slows neurodegeneration in prion infection
Patricia Aguilar-Calvo,
Adela Malik,
Daniel R. Sandoval
et al.
Abstract:Select prion diseases are characterized by widespread cerebral plaque-like deposits of amyloid fibrils enriched in heparan sulfate (HS), a major extracellular matrix component. HS facilitates fibril formation in vitro, yet how HS impacts fibrillar plaque growth within the brain is unclear. Here we found that prion-bound HS chains are highly sulfated, and that the sulfation is essential for accelerating prion conversion in vitro. Using conditional knockout mice to deplete the HS sulfation enzyme, Ndst1 (N-deace… Show more
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