Expanded polyglutamine embedded in the endoplasmic reticulum causes membrane distortion and coincides with Bax insertion.
Biochem Biophys Res Commun
; 474(2): 259-263, 2016 05 27.
Article
in En
| MEDLINE
| ID: mdl-27079237
ABSTRACT
The endoplasmic reticulum (ER) is important in various cellular functions, such as secretary and membrane protein biosynthesis, lipid synthesis, and calcium storage. ER stress, including membrane distortion, is associated with many diseases such as Huntington's disease. In particular, nuclear envelope distortion is related to neuronal cell death associated with polyglutamine. However, the mechanism by which polyglutamine causes ER membrane distortion remains unclear. We used electron microscopy, fluorescence protease protection assay, and alkaline treatment to analyze the localization of polyglutamine in cells. We characterized polyglutamine embedded in the ER membrane and noted an effect on morphology, including the dilation of ER luminal space and elongation of ER-mitochondria contact sites, in addition to the distortion of the nuclear envelope. The polyglutamine embedded in the ER membrane was observed at the same time as Bax insertion. These results demonstrated that the ER membrane may be a target of polyglutamine, which triggers cell death through Bax.
Key words
Full text:
1
Database:
MEDLINE
Main subject:
Peptides
/
Cell Membrane
/
Endoplasmic Reticulum
/
Bcl-2-Associated X Protein
/
Membrane Fluidity
Type of study:
Etiology_studies
Limits:
Humans
Language:
En
Year:
2016
Type:
Article