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Hypothetical pathway of polyGln-mediated aggregation and inclusion formation. Unstructured polyGln monomer undergoes a structural conversion to β-sheet, resulting in the formation of protofibrillar intermediates. This step may proceed through a linear growth mechanism or through assembly of oligomeric intermediates. Protofibril assembly is followed by fibril formation, resulting in the characteristic inclusions observed in polyGln diseases and other amyloid-like diseases.
