Protein clearance mechanisms and their demise in age-related neurodegenerative diseases

Isabel Saez; David Vilchez; Isabel Saez; David Vilchez

doi:10.3934/molsci.2015.1.1

AIMS Molecular Science

2015, Volume 1, Issue 1: 1-21. doi: 10.3934/molsci.2015.1.1

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Research article Special Issues

Protein clearance mechanisms and their demise in age-related neurodegenerative diseases

Isabel Saez ,
David Vilchez ^,

Cologne Excellence Cluster for Cellular Stress Responses in Aging-Associated Diseases (CECAD), University of Cologne, Joseph Stelzmann Strasse 26, 50931 Cologne, Germany

Received: 16 October 2014 Accepted: 18 December 2014 Published: 04 January 2015

The accumulation of damaged proteins contributes to the etiology of neurodegenerative diseases such as Alzheimer's, Parkinson's, Huntington's or amyotrophic lateral sclerosis. Protein aggregation and deposition are common features of these disorders that share emergence patterns and are more frequent late in life, even though different toxic proteins are involved in their onset. The ability to maintain a functional proteome, or proteostasis, declines during the ageing process. Damaged proteins are degraded by the ubiquitin proteasome system or through autophagy-lysosome, key components of the proteostasis network. Here we review the links between neurodegenerative disorders and loss of protein clearance mechanisms with age.
- proteostasis,
- ageing,
- neurodegeneration,
- proteasome,
- autophagy
Citation: Isabel Saez, David Vilchez. Protein clearance mechanisms and their demise in age-related neurodegenerative diseases[J]. AIMS Molecular Science, 2015, 1(1): 1-21. doi: 10.3934/molsci.2015.1.1

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Abstract

The accumulation of damaged proteins contributes to the etiology of neurodegenerative diseases such as Alzheimer's, Parkinson's, Huntington's or amyotrophic lateral sclerosis. Protein aggregation and deposition are common features of these disorders that share emergence patterns and are more frequent late in life, even though different toxic proteins are involved in their onset. The ability to maintain a functional proteome, or proteostasis, declines during the ageing process. Damaged proteins are degraded by the ubiquitin proteasome system or through autophagy-lysosome, key components of the proteostasis network. Here we review the links between neurodegenerative disorders and loss of protein clearance mechanisms with age.

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