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Nonetheless, in two preclinical models of cancer cachexia, the activation of several markers of ER stress (e.g., IRE1a, XBP-1, and ATF6) was accompanied by the suppression of mTORC1 signaling, activation of protein breakdown, and dysregulated AMPK activation .
The gene expression of GRP78, ATF6, and CHOP, the markers of ER stress, was upregulated only in the PA stimulation group compared to OA treatment group (Figure 3(a)).
The UPR pathways are activated by three ER stress sensors located on the ER membrane, namely, IRE1, ATF6, and PERK.
Furthermore, some of these proteins alter the activity of the UPR stress sensors (IRE1[alpha], PERK, and ATF6) as well as the activity/levels of downstream signaling mediators and transcription factors, including cleaved ATF6, ATF4, and spliced XBP1.
Trouve, "Coupling cystic fibrosis to endoplasmic reticulum stress: differential role of Grp78 and ATF6," Biochimica et Biophysica Acta: Molecular Basis of Disease, vol.
Prywes, "Dependence of site-2 protease cleavage of ATF6 on prior site-1 protease digestion is determined by the size of the luminal domain of ATF6," The Journal of Biological Chemistry, vol.
However, nuclear factor-kappa B (NF-[kappa]B), Jun N-terminal kinase (JNK), and activating transcription factor 6 (ATF6) are considered crucial signaling pathways for ERS-initiated inflammation [9, 13].
For core-dependent induction of ER stress it was described that both the EIF2AK3 and ATF6 pathways of the unfolded protein response (UPR) were activated by HCV core protein.
When an organism is exposed to ER stress, UPR-related pathways are activated by three sensor proteins which exist in the ER: activating transcription factor-6 (ATF6), protein kinase R (PKR)-like endoplasmic reticulum kinase (PERK), and inositol requiring enzyme 1a (IRE1[alpha]) .
In addition, reduction in endoplasmic reticulum stress was due to the significant downregulation of glucose-regulated protein (GRP78), phosphorylated protein kinase RNA-like endoplasmic reticulum kinase (p-PERK), activating transcription factor 4 (ATF4) and activating transcription factor 6 (ATF6)."
A type of achromatopsia previously identified as ACHM1 was later found to be the same as ACHM3 caused by cyclic nucleotide-gated channel beta-3 (CNGB3) gene (MIM 605080).8 Among other genes are included cyclic nucleotide-gated channel alpha-3 (CNGA3; MIM 600053) causing ACHM29, Guanine nucleotide-binding protein G subunit [alpha]-2 (GNAT2; MIM 139340) causing ACHM4, 10 phosphodiesterase 6C (PDE6C; MIM 613093) causing ACHM5, 11 cone inhibitory phosphodiesterase 6H (PDE6H; MIM 601190) causing ACHM612 and activating transcription factor 6 (ATF6; MIM 605537) causing ACHM7.13 The current study was planned to report two Pakistani families that were initially evaluated as having nystagmus phenotype.
The signaling pathway in ER stress is mediated by three major ER stress sensors: PKR-like ER kinase (PERK), inositol requiring enzyme 1 (IRE1), and activating transcription factor 6 (ATF6).
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