Protein Aggregation and Human Disorders

Robert D Moir, Massachusetts General Hospital and Harvard Medical School, Charlestown, Massachusetts, USA
Rudolph E Tanzi, Massachusetts General Hospital and Harvard Medical School, Charlestown, Massachusetts, USA

Published online: January 2006

DOI: 10.1038/npg.els.0005496

The deposition of insoluble proteinaceous material is a distinct pathological process associated with a broad range of disparate diseases. Modulating the self-aggregation of the proteins that form these deposits has emerged as a major therapeutic strategy for disease treatment.

Keywords: aggregation; amyloid; prion; fibrils; polyglutamine repeat

 References
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 Further Reading
    book Benson MD (1993) "Hereditary amyloidosis". In: Conneally PM (ed.) Molecular Basis of Neurology, 1st edn. Boston, MA: Blackwell Scientific Publications.
    Dobson CM (2001) The structural basis of protein folding and its links with human disease. Philosophical Transactions of the Royal Society of London B Biological Science 356: 133–145.
    Prusiner SB (2001) Shattuck lecture – neurodegenerative diseases and prions. New England Journal of Medicine 344: 1516–1526.
    book Raso SW and King JK (1997) "Protein folding and human disease". In: Pain RH (ed.) Mechanisms of Protein Folding, 2nd edn, pp. 406–428. New York, NY: Oxford University Press.
    book Tanzi RE and Parson AB (2000) Decoding Darkness, 1st edn. Cambridge, UK: Perseus Books.
    book Wetzel RE (1999) Amyloid, Prions and Other Protein Aggregates. New York, NY: Academic Press.

Related Sub-Topics:

Protein Folding, Evolution and Degradation | Specific Genetic Disorders | Proteomic Studies of Genetic Disease | Posttranslational Modification | Mechanisms of Cell Death, Senescence and Metabolism
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Article Title: Protein Aggregation and Human Disorders
 
How to Cite close
Moir, Robert D, and Tanzi, Rudolph E(Jan 2006) Protein Aggregation and Human Disorders. In: eLS. John Wiley & Sons Ltd, Chichester. http://www.els.net [doi: 10.1038/npg.els.0005496]