Mechanisms of protein-folding diseases at a glance
For a protein to function appropriately, it must first achieve its proper conformation and location within the crowded environment inside the cell. Multiple chaperone systems are required to fold proteins correctly. In addition, degradation pathways participate by destroying improperly folded protei...
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The Company of Biologists
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doaj-f60cd97f4486401a8c9281b91790f4cf2020-11-24T21:46:25ZengThe Company of BiologistsDisease Models & Mechanisms1754-84031754-84112014-01-017191410.1242/dmm.013474013474Mechanisms of protein-folding diseases at a glanceJulie S. ValastyanSusan LindquistFor a protein to function appropriately, it must first achieve its proper conformation and location within the crowded environment inside the cell. Multiple chaperone systems are required to fold proteins correctly. In addition, degradation pathways participate by destroying improperly folded proteins. The intricacy of this multisystem process provides many opportunities for error. Furthermore, mutations cause misfolded, nonfunctional forms of proteins to accumulate. As a result, many pathological conditions are fundamentally rooted in the protein-folding problem that all cells must solve to maintain their function and integrity. Here, to illustrate the breadth of this phenomenon, we describe five examples of protein-misfolding events that can lead to disease: improper degradation, mislocalization, dominant-negative mutations, structural alterations that establish novel toxic functions, and amyloid accumulation. In each case, we will highlight current therapeutic options for battling such diseases.http://dmm.biologists.org/content/7/1/9DiseaseMisfoldingProteinYeast |
collection |
DOAJ |
language |
English |
format |
Article |
sources |
DOAJ |
author |
Julie S. Valastyan Susan Lindquist |
spellingShingle |
Julie S. Valastyan Susan Lindquist Mechanisms of protein-folding diseases at a glance Disease Models & Mechanisms Disease Misfolding Protein Yeast |
author_facet |
Julie S. Valastyan Susan Lindquist |
author_sort |
Julie S. Valastyan |
title |
Mechanisms of protein-folding diseases at a glance |
title_short |
Mechanisms of protein-folding diseases at a glance |
title_full |
Mechanisms of protein-folding diseases at a glance |
title_fullStr |
Mechanisms of protein-folding diseases at a glance |
title_full_unstemmed |
Mechanisms of protein-folding diseases at a glance |
title_sort |
mechanisms of protein-folding diseases at a glance |
publisher |
The Company of Biologists |
series |
Disease Models & Mechanisms |
issn |
1754-8403 1754-8411 |
publishDate |
2014-01-01 |
description |
For a protein to function appropriately, it must first achieve its proper conformation and location within the crowded environment inside the cell. Multiple chaperone systems are required to fold proteins correctly. In addition, degradation pathways participate by destroying improperly folded proteins. The intricacy of this multisystem process provides many opportunities for error. Furthermore, mutations cause misfolded, nonfunctional forms of proteins to accumulate. As a result, many pathological conditions are fundamentally rooted in the protein-folding problem that all cells must solve to maintain their function and integrity. Here, to illustrate the breadth of this phenomenon, we describe five examples of protein-misfolding events that can lead to disease: improper degradation, mislocalization, dominant-negative mutations, structural alterations that establish novel toxic functions, and amyloid accumulation. In each case, we will highlight current therapeutic options for battling such diseases. |
topic |
Disease Misfolding Protein Yeast |
url |
http://dmm.biologists.org/content/7/1/9 |
work_keys_str_mv |
AT juliesvalastyan mechanismsofproteinfoldingdiseasesataglance AT susanlindquist mechanismsofproteinfoldingdiseasesataglance |
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1725902331579465728 |