cerebral protein aggregation

Aggregation of proteins in the brain is a common theme in a diverse group of disorders. The aggregation of tau (MAPT) in Alzheimer disease and various tauopathies is but one example of abnormal protein-protein interactions that result in the intracellular accumulation of filamentous proteins.

Abnormal protein aggregation is observed in a large number of neurodegenerative disorders:

 Alzheimer disease (AD) is characterized by the extracellular accumulation of Aβ fibrils in the form of amyloid plaques;
 Lewy body disorders contain intracytoplasmic filamentous aggregates of α-synuclein;
 trinucleotide repeat disorders have intranuclear inclusions composed of fibrous polyglutamines;
 spongiform encephalopathies demonstrate aggregates of proteinase-resistant prion protein.

Localization

 neuronal protein aggregates
 glial protein aggregates

See also

 neurofibrillary tangles (NFTs)
 protein aggregates

• extraneuronal protein aggregates

References

 Caughey B, Lansbury PT. Protofibrils, pores, fibrils, and neurodegeneration: separating the responsible protein aggregates from the innocent bystanders. Annu Rev Neurosci. 2003;26:267-98. PMID: 12704221