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If the UPR system fails to correct the insult, apoptotic programmes are activated.
Dysregulation of the UPR system instigates the occurrence of oxidative stress consequently resulting in the induction of apoptosis.
With prolonged absence or misfolding of one component of the USH complex, the UPR system could be overwhelmed, leading to ER stress.
However, when the UPR system is compromised, for example, by genetic haploinsufficiency of XBP1, aminoglycoside-induced mistranslation can manifest as neuropathology.
The UPR system is involved in balancing the load of newly synthesized proteins with the capacity of the ER to facilitate their maturation.
It is therefore not surprising that dysfunction of the UPR system leading to the loss of pyramidal cells via apoptosis may have occurred in our Tat-induced rat model of HAND because of the emerging importance of improper functioning of the UPR system in the development of neurodegenerative diseases [ 47].
Similar(53)
From work in other organisms, we anticipated the involvement of protein kinases, small GTPases and, in the case of the unfolded protein response (UPR), a system that responds to increased concentrations of non-native polypeptides within the endoplasmic reticulum, clear evidence for a transcriptional mechanism.
As expression and splicing of Xbp1 is regulated by the UPR stress response system or by the ER stress response, the effect of UPR signaling on IL-3 stimulation was investigated.
ER-stress activates the unfolded protein response (UPR), a homeostatic system that balances protein biosynthesis, folding and destruction, that eliminates cells experiencing excessive ER-stress via UPR-induced apoptosis.
In brief, UPR contains two systems involved in the attenuation of global translation to stop the influx of proteins into the ER and increasing the transcription of chaperones to refold the unfolded proteins in the ER again.
UPR is an alarm system for cells in response to the accumulation of unfolded or misfolded proteins in the lumen of the endoplasmic reticulum (ER), triggering formation of autophagy to digest and clean these unwanted proteins.
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