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Neuronal Ndst1 depletion accelerates prion protein clearance and slows neurodegeneration in prion infection

Fig 3

Reducing neuronal HS sulfation increases mCWD solubility and decreases fibril length.

(A) Representative western blot of mCWD aggregates after PK digestion and centrifugation over an Optiprep layer (P = pellet, S = supernatant). (B) Quantification of PrPSc in pellet and supernatant fractions. N = 6 samples per genotype. (C) Representative electron microscopy images of purified PrPSc fibrils from mCWD-infected mice. Scale bars represent 100 nm. Fibril measurements (bottom panel) are 193 nm (SynCre-) and 144 nm (SynCre+). (D) Quantification of the fibril lengths. N = 49 and 42 fibrils measured from SynCre- and SynCre+ brains, respectively (n = 6 mice per genotype). *P< 0.05, unpaired two-tailed t-test with Bonferroni’s post test (panels B and D).

Fig 3

doi: https://doi.org/10.1371/journal.ppat.1011487.g003