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NOX2 staining around the rim of a spongiform vacuole in prion disease.

NOX3 staining in brown, nuclei staining in blue. Spongiform vacuolation is a highly characteristic sign of prion disease pathology, and vacuoles represent distended intracellular compartments mostly located within neuronal processes. A very striking pattern of NOX2 expression around neuronal vacuoles can be detected in brains of patients affected by Creutzfeld-Jakob disease. Data from Adriano Aguzzi and colleagues suggest that NOX2 is a major source of reactive oxygen species in prion diseases, and can affect prion pathogenesis.

Image Credit: Silvia Sorce et al.