Abstract
Small heat shock proteins (sHsps) are molecular chaperones ubiquitously distributed in numerous species, from bacteria to humans. A conserved C-terminal “α-crystallin” domain organized in a β-sheet sandwich and oligomeric structure are common features of sHsps. sHsps protect cells against many kinds of stresses including heat shock, oxidative and osmotic stress. sHsps recognize unfolded proteins, prevent their irreversible aggregation and facilitate refolding of bound substrates in cooperation with ATP-dependent molecular chaperones (Hsp70/Hsp40). Mammalian sHsps (HSPBs) are multifunctional proteins involved in many cellular processes including those which are not directly related to protein folding and aggregation. HSPBs participate in cell development and cancerogenesis, regulate apoptosis and control cytoskeletal architecture. Recent data revealed that HSPBs also play an important role in membrane stabilization. Mutation in HSPB genes have been identified, which are responsible for the development of cataract, desmin related myopathy and neuropathies. HSPBs are often found as components of protein aggregates associated with protein-misfolding disorders, such as Parkinsons, Alzheimers, Alexanders and prion diseases. It is supposed that the presence of HSPBs in intra- or extracellular protein deposits is a consequence of the chaperone activity of HSPBs, however more studies are needed to reveal the exact function of HSPBs during the formation (or removal) of disease-related aggregates.
Keywords: Small heat shock proteins, molecular chaperones, protein aggregates, protein-misfolding diseases, cataract, neurodegenerative diseases
Current Pharmaceutical Biotechnology
Title: Small Heat Shock Proteins and Protein-Misfolding Diseases
Volume: 11 Issue: 2
Author(s): Ewa Laskowska, Ewelina Matuszewska and Dorota Kuczynska-Wisnik
Affiliation:
Keywords: Small heat shock proteins, molecular chaperones, protein aggregates, protein-misfolding diseases, cataract, neurodegenerative diseases
Abstract: Small heat shock proteins (sHsps) are molecular chaperones ubiquitously distributed in numerous species, from bacteria to humans. A conserved C-terminal “α-crystallin” domain organized in a β-sheet sandwich and oligomeric structure are common features of sHsps. sHsps protect cells against many kinds of stresses including heat shock, oxidative and osmotic stress. sHsps recognize unfolded proteins, prevent their irreversible aggregation and facilitate refolding of bound substrates in cooperation with ATP-dependent molecular chaperones (Hsp70/Hsp40). Mammalian sHsps (HSPBs) are multifunctional proteins involved in many cellular processes including those which are not directly related to protein folding and aggregation. HSPBs participate in cell development and cancerogenesis, regulate apoptosis and control cytoskeletal architecture. Recent data revealed that HSPBs also play an important role in membrane stabilization. Mutation in HSPB genes have been identified, which are responsible for the development of cataract, desmin related myopathy and neuropathies. HSPBs are often found as components of protein aggregates associated with protein-misfolding disorders, such as Parkinsons, Alzheimers, Alexanders and prion diseases. It is supposed that the presence of HSPBs in intra- or extracellular protein deposits is a consequence of the chaperone activity of HSPBs, however more studies are needed to reveal the exact function of HSPBs during the formation (or removal) of disease-related aggregates.
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Cite this article as:
Laskowska Ewa, Matuszewska Ewelina and Kuczynska-Wisnik Dorota, Small Heat Shock Proteins and Protein-Misfolding Diseases, Current Pharmaceutical Biotechnology 2010; 11 (2) . https://dx.doi.org/10.2174/138920110790909669
DOI https://dx.doi.org/10.2174/138920110790909669 |
Print ISSN 1389-2010 |
Publisher Name Bentham Science Publisher |
Online ISSN 1873-4316 |
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