scholarly journals PrP-containing aggresomes are cytosolic components of an ER quality control mechanism

2016 ◽  
Vol 129 (19) ◽  
pp. 3635-3647 ◽  
Author(s):  
Tatyana Dubnikov ◽  
Tziona Ben-Gedalya ◽  
Robert Reiner ◽  
Dominic Hoepfner ◽  
Wayne A. Cabral ◽  
...  
Keyword(s):  
Quality Control ◽  
Control Mechanism ◽  
Er Quality Control ◽  
Quality Control Mechanism

scholarly journals Peters Plus Syndrome Mutations Disrupt a Noncanonical ER Quality-Control Mechanism

2015 ◽  
Vol 25 (3) ◽  
pp. 286-295 ◽  
Author(s):  
Deepika Vasudevan ◽  
Hideyuki Takeuchi ◽  
Sumreet Singh Johar ◽  
Elaine Majerus ◽  
Robert S. Haltiwanger
Keyword(s):  
Quality Control ◽  
Control Mechanism ◽  
Er Quality Control ◽  
Quality Control Mechanism

scholarly journals Misfolded proteins are sorted by a sequential checkpoint mechanism of ER quality control

2004 ◽  
Vol 165 (1) ◽  
pp. 41-52 ◽  
Author(s):  
Shilpa Vashist ◽  
Davis T.W. Ng
Keyword(s):  
Quality Control ◽  
Membrane Proteins ◽  
Control Mechanism ◽  
Degradation Pathway ◽  
Misfolded Proteins ◽  
Er Quality Control ◽  
Golgi Transport ◽  
Test Face ◽  
Folded Proteins ◽  
Quality Control Mechanism

Misfolded proteins retained in the endoplasmic reticulum (ER) are degraded by the ER-associated degradation pathway. The mechanisms used to sort them from correctly folded proteins remain unclear. Analysis of substrates with defined folded and misfolded domains has revealed a system of sequential checkpoints that recognize topologically distinct domains of polypeptides. The first checkpoint examines the cytoplasmic domains of membrane proteins. If a lesion is detected, it is retained statically in the ER and rapidly degraded without regard to the state of its other domains. Proteins passing this test face a second checkpoint that monitors domains localized in the ER lumen. Proteins detected by this pathway are sorted from folded proteins and degraded by a quality control mechanism that requires ER-to-Golgi transport. Although the first checkpoint is obligatorily directed at membrane proteins, the second monitors both soluble and membrane proteins. Our data support a model whereby “properly folded” proteins are defined biologically as survivors that endure a series of distinct checkpoints.

scholarly journals Quality-Control Mechanism for Telomerase RNA Folding in the Cell

Cell Reports ◽  
2020 ◽  
Vol 33 (13) ◽  
pp. 108568
Author(s):  
Xichan Hu ◽  
Jin-Kwang Kim ◽  
Clinton Yu ◽  
Hyun-Ik Jun ◽  
Jinqiang Liu ◽  
...  
Keyword(s):  
Quality Control ◽  
Rna Folding ◽  
Control Mechanism ◽  
Telomerase Rna ◽  
Quality Control Mechanism

Overexpression of Buffy enhances the loss of parkin and suppresses the loss of Pink1 phenotypes in Drosophila

Genome ◽  
2017 ◽  
Vol 60 (3) ◽  
pp. 241-247
Author(s):  
P. Githure M’Angale ◽  
Brian E. Staveley
Keyword(s):  
Gene Expression ◽  
Quality Control ◽  
Early Onset ◽  
Autosomal Recessive ◽  
Control Mechanism ◽  
Nigrostriatal Neurons ◽  
Climbing Ability ◽  
Quality Control Mechanism ◽  
Locomotor Ability ◽  
Early Onset Parkinson’S Disease

Mutations in parkin (PARK2) and Pink1 (PARK6) are responsible for autosomal recessive forms of early onset Parkinson’s disease (PD). Attributed to the failure of neurons to clear dysfunctional mitochondria, loss of gene expression leads to loss of nigrostriatal neurons. The Pink1/parkin pathway plays a role in the quality control mechanism aimed at eliminating defective mitochondria, and the failure of this mechanism results in a reduced lifespan and impaired locomotor ability, among other phenotypes. Inhibition of parkin or Pink1 through the induction of stable RNAi transgene in the Ddc-Gal4-expressing neurons results in such phenotypes to model PD. To further evaluate the effects of the overexpression of the Bcl-2 homologue Buffy, we analysed lifespan and climbing ability in both parkin-RNAi- and Pink1-RNAi-expressing flies. In addition, the effect of Buffy overexpression upon parkin-induced developmental eye defects was examined through GMR-Gal4-dependent expression. Curiously, Buffy overexpression produced very different effects: the parkin-induced phenotypes were enhanced, whereas the Pink1-enhanced phenotypes were suppressed. Interestingly, the overexpression of Buffy along with the inhibition of parkin in the neuron-rich eye results in the suppression of the developmental eye defects.

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