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Endoplasmic Reticulum Protein Quality Control Failure in Myelin Disorders
Vera G Volpi1, Thierry Touvier1, Maurizio D'Antonio1
1Biology of Myelin Unit, Division of Genetics and Cell Biology, San Raffaele Scientific Institute, DIBIT Milan, Italy.
Frontiers in Molecular Neuroscience
|January 20, 2017
Summary
Proper protein folding in the endoplasmic reticulum (ER) is vital for cell function. ER quality control (ERQC) systems maintain protein homeostasis, and their failure is linked to myelin disorders.
Area of Science:
- Cell Biology
- Neuroscience
- Biochemistry
Background:
- Protein folding and maturation occur in the endoplasmic reticulum (ER).
- The ER-protein quality control (ERQC) system ensures protein homeostasis by assisting folding and degrading misfolded proteins.
- The unfolded protein response (UPR) is activated by ER stress and can lead to cell death if unresolved.
Conclusions:
- Proper ERQC function is critical for preventing myelin disorders.
- ER stress and UPR play significant roles in the pathogenesis of these neurological conditions.
- Understanding ERQC mechanisms offers insights into potential therapeutic strategies for myelin diseases.
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