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Transmissible cerebral amyloidoses as a model for Alzheimer’s disease

An ultrastructural perspective

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Abstract

Alzheimer’s disease, a prototypic nontransmissible cerebral amyloidosis, has no adequate experimental model. Several pathogenetic events, however, may be modeled and accurately studied in the transmissible cerebral amyloidoses of kuru, Creutzfeldt-Jakob disease, Gerstmann-Sträussler-Scheinker disease, and scrapie. The common neuropathological denominator in both types of cerebral amyloidoses is the presence of stellate kuru plaques, senile plaques, and pure neuritic plaques. These amyloid plaques consist of amyloid fibers, dystrophic neurites, and reactive astrocytes in different proportions. Microglial cells, which are regarded as amyloid producer/processor cells in Alzheimer’s disease, may play the same function in the transmissible cerebral amyloidoses. In both transmissible and nontransmissible amyloidoses, the impairment of axonal transport leads to accumulation of abnormally phosphorylated cytoskeleton proteins (such as neurofilament proteins and microtubule-associated protein τ), which eventually produce dystophic neurites observed as parts of plaque or as isolated pathological structures.

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  1. Electron Microscopic Laboratory, Department of Oncology, Medical Academy Lodz, Paderewskiego 4, PL 93-509, Lodz, Poland

    Pawel Piotr Liberski

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Liberski, P.P. Transmissible cerebral amyloidoses as a model for Alzheimer’s disease. Mol Neurobiol 8, 67–77 (1994). https://doi.org/10.1007/BF02778009

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