NTU Undergraduates' research April 2014 - Biosciences | Page 30

Abstract
This review summarises the current field of prions including our understanding of the
underlying mechanisms involved in the development of neurodegenerative disorders
alongside current treatments. Transmissible spongiform encephlopathies (TSEs), also known
as prion diseases, describe a group of these neurodegenerative disorders affecting animals
and humans. Formation of protein amyloid aggregates leading to accumulation of misfolded
protein is thought to be the event greatly responsible for the manifestation of these disorders.
Computational models have assisted in the understanding of protein folding by recreating
protein folding events that have allowed the mechanism of aggregation of protein into
fibrillar species to surface. This article describes the pathways involved in protein folding,
aggregation and self-assembly of proteins including the consequences involved when
aberrant folding occurs. Therapeutic strategies in treating patients with prion disease are also
discussed.