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Targeting defective proteostasis in the collagenopathies
Name
nihms-1523420.pdf
Description
Accepted version
Size
1.28 MB
Format
Adobe PDF
Checksum (MD5)
d3a60d259ed437438b7a88a22e304f4d
Author(s)
Wong, Madeline Y
Shoulders, Matthew D
Journal
Current Opinion in Chemical Biology
Publisher
Elsevier BV
Version
Author's final manuscript
Abstract
© 2019 Elsevier Ltd The collagenopathies are a diverse group of diseases caused primarily by mutations in collagen genes. The resulting disruptions in collagen biogenesis can impair development, cause cellular dysfunction, and severely impact connective tissues. Most existing treatment options only address patient symptoms. Yet, while the disease-causing genes and proteins themselves are difficult to target, increasing evidence suggests that resculpting the intracellular proteostasis network, meaning the machineries responsible for producing and ensuring the integrity of collagen, could provide substantial benefit. We present a proteostasis-focused perspective on the collagenopathies, emphasizing progress toward understanding how mechanisms of collagen proteostasis are disrupted in disease. In parallel, we highlight recent advances in small molecule approaches to tune endoplasmic reticulum proteostasis that may prove useful in these disorders.
Terms of Use
Creative Commons Attribution-NonCommercial-NoDerivs License
Persistent DSpace Link
DOI of Published Version
10.1016/J.CBPA.2019.02.021
