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Sick chaperones and ageing: a perspective
Alberto J L Macario1, Everly Conway de Macario
1Wadsworth Center, Division of Molecular Medicine, New York State Department of Health, Empire State Plaza, P.O. Box 509, Albany, New York 12201-0509, USA. macario@wadsworth.org
Ageing Research Reviews
|June 1, 2002
Summary
Proteinopathies involve abnormal protein deposits. This article introduces chaperonepathies, a subset caused by defective molecular chaperones, impacting aging and disease.
Area of Science:
- Molecular biology
- Cellular biology
- Pathology
Background:
- Proteinopathies are diseases characterized by intra- and extra-cellular protein deposits.
- These deposits often involve defective proteins that misfold and aggregate.
- In many proteinopathies, molecular chaperones and their systems are normal.
Purpose of the Study:
- To introduce and define a subset of proteinopathies termed chaperonepathies.
- To explore the role of defective chaperones in disease pathogenesis.
- To investigate the link between chaperone dysfunction, senescence, and aging.
Main Methods:
- Review of existing literature on proteinopathies and molecular chaperones.
- Conceptual framework development for chaperonepathies.
- Analysis of the impact of defective chaperones on cellular systems and organismal health.
Main Results:
- Chaperonepathies are disorders where defective chaperones are the primary cause.
- Defective chaperones have widespread deleterious effects due to their ubiquitous nature.
- Chaperone dysfunction contributes to senescence and accelerated aging, especially when combined with stress.
Conclusions:
- Chaperonepathies represent a distinct category of proteinopathies.
- Defective chaperones are implicated in clinically heterogeneous diseases.
- Chaperone system failure is a significant factor in aging and age-associated decline.