Figure 5

Histopathological phenotypes and lesion profiles. (A) Hematoxylin and eosin (i and ii) and PrP immunohistochemistry (iii). i-iii (1^st and 2^nd rows), i: Spongiform degeneration (SD) affecting only deep cortical layers (II: layer 2; V: layer 5); ii: Small vacuoles SD; iii: Plaque-like PrP immunostaining in the granular layer (Grl. L.) of the cerebellum; Mol. L: molecular layer; i-iii (3^rd-5^th rows); i: SD with medium size vacuoles, scattered ballooned neurons and atrophy affecting all layers of the cerebral cortex; insets in i (rows 3^rd and 5^th): a ballooned neuron. iii: Presence and lack (3^rd, 4^th and 5^th rows, respectively) of plaque-like PrP in the granular layer. Scale bar insets: 25 µm. (B,C) Lesion profiles. (B) The lesion profile of all -VV1-2 cases combined resembled that of -VV2 except for the cerebellum (CE). (C) Lesion profiles of -VV1-2 with low (≤9%; cases 15-18, Table S2), intermediate (37–77%; cases 9-11, 13, 14) and high (≥91%; cases 1-8) representation of T2. The -VV1-2 lesion profile shifts progressively from resembling -VV1 at low %T2 to virtually coincide with -VV2 at high %T2. Each point of the lesion profile is expressed as mean ± SEM of severity scores related to spongiosis and gliosis. FC: frontal cortex (cx); TC: temporal cx; PC: parietal cx; OC: occipital cx; HI: hippocampus (CA1 region); EC: entorhinal cortex; BG: basal ganglia; TH: thalamus, SN: substantia nigra; CE: cerebellum. *P < 0.05-0.04; ***P < 0.0006-0.0001.