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Buchner, Johannes ; Scheibel, Thomas:
Protein Aggregation as a Cause for Disease.
In: Starke, Br. K. ; Gaestel, Matthias
(Hrsg.):
Molecular chaperones in Health and Disease. -
Berlin
: Springer
,
2006
. - S. 199-219
. - (Handbook of Experimental Pharmacology
; 172
)
ISBN 978-3-540-25875-9
DOI: https://doi.org/10.1007/3-540-29717-0_9
Abstract
The ability of proteins to fold into a defined and functional conformation is one of the most fundamental processes in biology. Certain conditions, however, initiate misfolding or unfolding of proteins. This leads to the loss of functional protein or it can result in a wide range of diseases. One group of diseases, which includes Alzheimer’s, Parkinson’s, Huntington’s disease, and the transmissible spongiformencephalopathies (prion diseases),
involves deposition of aggregated proteins. Normally, such protein aggregates are not found in properly functioning biological systems, because a variety of mechanisms inhibit their formation. Understanding the nature of these protective mechanisms together with the understanding of factors reducing or deactivating the natural protection machinery will be crucial for developing strategies to prevent and treat these disastrous diseases.