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PART 17: Neurologic Disorders
SECTION 3   Diseases of the Central Nervous System

383 Prion Diseases
Stanley B. Prusiner / Bruce L. Miller

Figure 383-1  Structures of prion proteins. A. NMR structure of Syrian hamster recombinant (rec) PrP(90–231). Presumably, the structure of the α-helical form of recPrP(90–231) resembles that of PrPC. recPrP(90–231) is viewed from the...
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Figure 383-2  Prion protein isoforms. Bar diagram of Syrian hamster PrP, which consists of 254 amino acids. After processing of the NH2 and COOH termini, both PrPC and PrPSc consist of 209 residues. After limited proteolysis, the NH2 terminus of PrPSc is...
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Figure 383-3  T2-weighted (FLAIR) MRI showing hyperintensity in the cortex in a patient with sporadic CJD. This so-called “cortical ribboning” along with increased intensity in the basal ganglia on T2 or diffusion-weighted imaging can aid in the diagnosis of CJD.
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