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ER-associated degradation

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https://www.readbyqxmd.com/read/28437467/mutant-uromodulin-expression-leads-to-altered-homeostasis-of-the-endoplasmic-reticulum-and-activates-the-unfolded-protein-response
#1
Céline Schaeffer, Stefania Merella, Elena Pasqualetto, Dejan Lazarevic, Luca Rampoldi
Uromodulin is the most abundant urinary protein in physiological conditions. It is exclusively produced by renal epithelial cells lining the thick ascending limb of Henle's loop (TAL) and it plays key roles in kidney function and disease. Mutations in UMOD, the gene encoding uromodulin, cause autosomal dominant tubulointerstitial kidney disease uromodulin-related (ADTKD-UMOD), characterised by hyperuricemia, gout and progressive loss of renal function. While the primary effect of UMOD mutations, retention in the endoplasmic reticulum (ER), is well established, its downstream effects are still largely unknown...
2017: PloS One
https://www.readbyqxmd.com/read/28428258/deletion-of-inositol-requiring-enzyme-1%C3%AE-in-podocytes-disrupts-glomerular-capillary-integrity-and-autophagy
#2
Daniel Robert Kaufman, Joan Papillon, Louise Larose, Takao Iwawaki, Andrey V Cybulsky
Inositol-requiring enzyme-1α (IRE1α) is an endoplasmic reticulum (ER)-transmembrane endoribonuclease-kinase, which plays an essential function in extraembryonic tissues during normal development, and is activated during ER stress. To address the functional role of IRE1α in glomerular podocytes, we produced podocyte-specific IRE1α deletion mice. In male mice, deletion of IRE1α in podocytes resulted in albuminuria beginning at 5 months of age, and worsening with time. Electron microscopy revealed focal podocyte foot process effacement in 9-month old male IRE1α deletion mice, as well as microvillous transformation of podocyte plasma membranes...
April 20, 2017: Molecular Biology of the Cell
https://www.readbyqxmd.com/read/28425050/oxidative-protein-modification-alters-proteostasis-under-acute-hypobaric-hypoxia-in-skeletal-muscles-a-comprehensive-in-vivo-study
#3
Akanksha Agrawal, Richa Rathor, Geetha Suryakumar
While numerous maladies are associated with hypobaric hypoxia, muscle protein loss is an important under studied topic. Hence, the present study was designed to investigate the mechanism of muscle protein loss at HH. SD rats were divided into normoxic rats, while remaining rats were exposed to simulated hypoxia equivalent to 282-torr pressure (equal to an altitude of 7620 m, 8% oxygen), at 25 °C for 6, 12, and 24 h. Post-exposure rats were sacrificed and analysis was performed. Ergo, muscle loss-related changes were observed at 12 and 24 h post-HH exposure...
April 19, 2017: Cell Stress & Chaperones
https://www.readbyqxmd.com/read/28424968/visualization-of-reticulophagy-in-living-cells-using-an-endoplasmic-reticulum-targeted-p62-mutant
#4
Liang Wang, Lei Liu, Lingsong Qin, Qingming Luo, Zhihong Zhang
Reticulophagy is a type of selective autophagy in which protein aggregate-containing and/or damaged endoplasmic reticulum (ER) fragments are engulfed for lysosomal degradation, which is important for ER homeostasis. Several chemical drugs and mutant proteins that promote protein aggregate formation within the ER lumen can efficiently induce reticulophagy in mammalian cells. However, the exact mechanism and cellular localization of reticulophagy remain unclear. In this report, we took advantage of the self-oligomerization property of p62/SQSTM1, an adaptor for selective autophagy, and developed a novel reticulophagy system based on an ER-targeted p62 mutant to investigate the process of reticulophagy in living cells...
April 13, 2017: Science China. Life Sciences
https://www.readbyqxmd.com/read/28419579/erdj3-has-an-essential-role-for-z-variant-alpha-1-antitrypsin-degradation
#5
Nazli Khodayari, George Marek, Yuanqing Lu, Karina Krotova, Rejean Liqun Wang, Mark Brantly
Alpha-1-antitrypsin deficiency (AATD) is an inherited disease characterized by emphysema and liver disease. AATD is most often caused by a single amino acid substitution at amino acid 342 in the mature protein, resulting in the Z mutation of the alpha-1-antitrypsin gene (ZAAT). This substitution is associated with misfolding and accumulation of ZAAT in the endoplasmic reticulum (ER) of hepatocytes and monocytes, causing a toxic gain of function. Retained ZAAT is eliminated by ER-associated degradation and autophagy...
April 17, 2017: Journal of Cellular Biochemistry
https://www.readbyqxmd.com/read/28419469/the-endoplasmic-reticulum-associated-protein-os-9-behaves-as-a-lectin-in-targeting-the-immature-calcium-sensing-receptor
#6
Bryan K Ward, Sarah L Rea, Aaron L Magno, Bernadette Pedersen, Suzanne J Brown, Shelby Mullin, Ajanthy Arulpragasam, Evan Ingley, Arthur D Conigrave, Thomas Ratajczak
The mechanisms responsible for the processing and quality control of the calcium-sensing receptor (CaSR) in the endoplasmic reticulum (ER) are largely unknown. In a yeast two-hybrid screen of the CaSR C-terminal tail (residues 865 -1078), we identified osteosarcoma-9 (OS-9) protein as a binding partner. OS-9 is an ER-resident lectin that targets misfolded glycoproteins to the ER-associated degradation (ERAD) pathway through recognition of specific N-glycans by its mannose-6-phosphate receptor homology (MRH) domain...
April 17, 2017: Journal of Cellular Physiology
https://www.readbyqxmd.com/read/28418178/unfolded-protein-response-activation-compensates-er-associated-degradation-deficiency-in-arabidopsis
#7
Qingliang Li, Hai Wei, Lijing Liu, Xiaoyuan Yang, Xiansheng Zhang, Qi Xie
Abiotic stresses often disrupt protein folding and induce endoplasmic reticulum (ER) stress. There is a sophisticated ER quality control (ERQC) system to mitigate the effects of malfunctioning proteins and maintain ER homeostasis. The accumulation of misfolded proteins in the ER activates the unfolded protein response (UPR) to enhance ER protein folding and the degradation of misfolded proteins mediate by ER-associated degradation (ERAD). That ERQC reduces abiotic stress damage has been well studied in mammals and yeast...
April 18, 2017: Journal of Integrative Plant Biology
https://www.readbyqxmd.com/read/28416613/hypoxia-inducible-factor-1%C3%AE-activates-insig-2-transcription-for-degradation-of-hmg-coa-reductase-in-the-liver
#8
Seonghwan Hwang, Andrew D Nguyen, Youngah Jo, Luke J Engelking, James Brugarolas, Russell A DeBose-Boyd
Cholesterol synthesis is a highly oxygen-consuming process. As such, oxygen deprivation (hypoxia) limits cholesterol synthesis through incompletely understood mechanisms mediated by the oxygen-sensitive transcription factor hypoxia-inducible factor (HIF)-1α. We show here that HIF-1α links pathways for oxygen sensing and feedback control of cholesterol synthesis in human fibroblasts by directly activating transcription of the INSIG-2 gene. Insig-2 is one of two endoplasmic reticulum (ER) membrane proteins that inhibit cholesterol synthesis by mediating sterol-induced ubiquitination and subsequent ER-associated degradation of the rate-limiting enzyme in the pathway, HMG CoA reductase (HMGCR)...
April 17, 2017: Journal of Biological Chemistry
https://www.readbyqxmd.com/read/28414129/the-arabidopsis-endoplasmic-reticulum-associated-degradation-pathways-are-involved-in-the-regulation-of-heat-stress-response
#9
Lin-Mao Li, Shi-You Lü, Rong-Jun Li
The Cytosolic Protein Response (CPR) in the cytosol and the Unfolded Protein Response (UPR) and ER-associated degradation (ERAD) in the endoplasmic reticulum are major pathways of the cellular proteostasis network. However, despite years of effort, how these protein quality control systems coordinated in vivo remains largely unknown, particularly in plants. In this study, the roles of two evolutionarily conserved ERAD pathways (DOA10 and HRD1) in heat stress response were investigated through reverse genetic approaches in Arabidopsis...
April 14, 2017: Biochemical and Biophysical Research Communications
https://www.readbyqxmd.com/read/28411238/characterization-of-protein-complexes-of-the-endoplasmic-reticulum-associated-degradation-e3-ubiquitin-ligase-hrd1
#10
Jiwon Hwang, Christopher P Walczak, Thomas A Shaler, James A Olzmann, Lichao Zhang, Joshua E Elias, Ron R Kopito
Hrd1 is the core structural component of a large ER membrane-embedded protein complex that coordinates the destruction of folding-defective proteins in the early secretory pathway. Defining the composition, dynamics, and ultimately, the structure of the Hrd1 complex is a crucial step in understanding the molecular basis of glycoprotein quality control, but has been hampered by the lack of suitable techniques to interrogate this complex under native conditions. In this study we used genome editing to generate clonal HEK293 (Hrd1...
April 14, 2017: Journal of Biological Chemistry
https://www.readbyqxmd.com/read/28402954/chaperone-mediated-autophagy-compensates-for-impaired-macroautophagy-in-the-cirrhotic-liver-to-promote-hepatocellular-carcinoma
#11
Srinivas Chava, Christine Lee, Yucel Aydin, Partha K Chandra, Asha Dash, Milad Chedid, Swan N Thung, Krzysztof Moroz, Tong Wu, Nabeen C Nayak, Srikanta Dash
Macroautophagy and chaperone-mediated autophagy (CMA) represent two major lysosomal degradation processes and often compensate for one another to facilitate cell survival. The aim of this study was to determine whether these autophagy pathways could compensate for one another to promote HCC cell survival in the cirrhotic liver. Analysis of normal liver tissue showed no expression of glypican-3 or p62 proteins, suggesting that macroautophagy is the major contributor to autophagic flux under non-pathological conditions...
March 29, 2017: Oncotarget
https://www.readbyqxmd.com/read/28397763/endoplasmic-reticulum-stress-and-homeostasis-in-reproductive-physiology-and-pathology
#12
REVIEW
Elif Guzel, Sefa Arlier, Ozlem Guzeloglu-Kayisli, Mehmet Selcuk Tabak, Tugba Ekiz, Nihan Semerci, Kellie Larsen, Frederick Schatz, Charles Joseph Lockwood, Umit Ali Kayisli
The endoplasmic reticulum (ER), comprises 60% of the total cell membrane and interacts directly or indirectly with several cell organelles i.e., Golgi bodies, mitochondria and proteasomes. The ER is usually associated with large numbers of attached ribosomes. During evolution, ER developed as the specific cellular site of synthesis, folding, modification and trafficking of secretory and cell-surface proteins. The ER is also the major intracellular calcium storage compartment that maintains cellular calcium homeostasis...
April 8, 2017: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/28394250/ampk-and-vacuole-associated-atg14p-orchestrate-%C3%A2%C2%B5-lipophagy-for-energy-production-and-long-term-survival-under-glucose-starvation
#13
Arnold Young Seo, Pick-Wei Lau, Daniel Feliciano, Prabuddha Sengupta, Mark A Le Gros, Bertrand Cinquin, Carolyn A Larabell, Jennifer Lippincott-Schwartz
Dietary restriction increases the longevity of many organisms but the cell signaling and organellar mechanisms underlying this capability are unclear. We demonstrate that to permit long-term survival in response to sudden glucose depletion, yeast cells activate lipid-droplet (LD) consumption through micro-lipophagy (µ-lipophagy), in which fat is metabolized as an alternative energy source. AMP-activated protein kinase (AMPK) activation triggered this pathway, which required Atg14p. More gradual glucose starvation, amino acid deprivation or rapamycin did not trigger µ-lipophagy and failed to provide the needed substitute energy source for long-term survival...
April 10, 2017: ELife
https://www.readbyqxmd.com/read/28380444/caspase-12-degrades-ikba-protein-and-enhances-mmp-9-expression-in-human-nasopharyngeal-carcinoma-cell-invasion
#14
Wing-Keung Chu, Chih-Chin Hsu, Shiang-Fu Huang, Chia-Chi Hsu, Shu-Er Chow
Caspase-12 (Casp12), an inflammatory caspase, functions as a dominant-negative regulator of inflammatory responses and is associated with the signaling of apoptosis. However, the physiological function of Casp12 presented in cancer cells is still unclear. This study demonstrated that overexpression of Casp12 mediated IκBα degradation and significantly increased NF-κB activity. Exposure of human nasopharyngeal carcinoma (NPC) cells to phorbol-12-myristate-13-acetate (PMA) increased the levels of Casp12 and MMP-9 resulting in NPC cell invasion...
March 23, 2017: Oncotarget
https://www.readbyqxmd.com/read/28367085/functional-analysis-of-a-nonsyndromic-hearing-loss-associated-mutation-in-the-transmembrane-ii-domain-of-the-gjc3-gene
#15
Swee-Hee Wong, Wen-Hung Wang, Pin-Hua Chen, Shuan-Yow Li, Jiann-Jou Yang
In a previous study, we identified a novel missense mutation, p.W77S, in the GJC3 gene encoding connexin30.2/connexin31.3 (CX30.2/CX31.3) from patients with hearing loss. The functional alteration of CX30.2/CX31.3 caused by the p.W77S mutant of GJC3 gene, however, remains unclear. In the current study, our result indicated that the p.W77 is localized at the second membrane-spanning segments (TM2) and near border of the E1 domain of the CX30.2/CX31.3 protein and highly conserved (Conseq score = 8~9) in all species...
2017: International Journal of Medical Sciences
https://www.readbyqxmd.com/read/28365313/transcriptome-analysis-provides-insights-into-hepatic-responses-to-moderate-heat-stress-in-the-rainbow-trout-oncorhynchus-mykiss
#16
Yongjuan Li, Jinqiang Huang, Zhe Liu, Yanjing Zhou, Binpeng Xia, Yongjie Wang, Yujun Kang, Jianfu Wang
The rainbow trout is an economically important fish in the world. The limited stress tolerance of this species to high summer-like temperatures usually leads to mass mortality and great economic loss. However, there is limited information on the mechanisms underlying moderate heat responses in the liver of the rainbow trout. Here, we performed transcriptome profiling of rainbow trout liver under moderate heat stress by using the Hiseq™ 4000 sequencing platform. >277 million clean reads were obtained from 6 libraries and aligned against the rainbow trout genome...
March 29, 2017: Gene
https://www.readbyqxmd.com/read/28361033/clinical-implications-of-esr1-mutations-in-hormone-receptor-positive-advanced-breast-cancer
#17
REVIEW
Tomas Reinert, Everardo D Saad, Carlos H Barrios, José Bines
Hormone receptor-positive breast cancer is the most frequent breast cancer subtype. Endocrine therapy (ET) targeting the estrogen receptor (ER) pathway represents the main initial therapeutic approach. The major strategies include estrogen deprivation and the use of selective estrogen modulators or degraders, which show efficacy in the management of metastatic and early-stage disease. However, clinical resistance associated with progression of disease remains a significant therapeutic challenge. Mutations of the ESR1 gene, which encodes the ER, have been increasingly recognized as an important mechanism of ET resistance, with a prevalence that ranges from 11 to 39%...
2017: Frontiers in Oncology
https://www.readbyqxmd.com/read/28351922/mouse-mammary-tumor-virus-signal-peptide-uses-a-novel-p97-dependent-and-derlin-independent-retrotranslocation-mechanism-to-escape-proteasomal-degradation
#18
Hyewon Byun, Poulami Das, Houqing Yu, Alejandro Aleman, Mary M Lozano, Andreas Matouschek, Jaquelin P Dudley
Multiple pathogens, including viruses and bacteria, manipulate endoplasmic reticulum-associated degradation (ERAD) to avoid the host immune response and promote their replication. The betaretrovirus mouse mammary tumor virus (MMTV) encodes Rem, which is a precursor protein that is cleaved into a 98-amino-acid signal peptide (SP) and a C-terminal protein (Rem-CT). SP uses retrotranslocation for ER membrane extraction and yet avoids ERAD by an unknown mechanism to enter the nucleus and function as a Rev-like protein...
March 28, 2017: MBio
https://www.readbyqxmd.com/read/28350102/renin-angiotensin-system-activation-accelerates-atherosclerosis-in-experimental-renal-failure-by-promoting-endoplasmic-reticulum-stress-related-inflammation
#19
Jia Yang, Xi Zhang, Xinyi Yu, Weixue Tang, Hua Gan
In this study, we investigated the association between the renin-angiotensin system (RAS), endoplasmic reticulum (ER) stress and atherosclerosis (AS) in uremic apolipoprotein E knockout (apoE-/-) mice. Mild uremia was induced by a 5/6 nephrectomy (5/6 Nx) in 10-week-old apoE-/- mice. Four weeks after nephrectomy, the mice received losartan or no treatment for 16 weeks. Sham-operated mice served as the controls. We found that uremia accelerated AS at the aortic root. The activation of ER stress and the significant upregulation of pro-inflammatory cytokines and chemokines were observed in the uremic mice...
January 12, 2017: International Journal of Molecular Medicine
https://www.readbyqxmd.com/read/28341998/ire1-signaling-exacerbates-alzheimer-s-disease-pathogenesis
#20
Claudia Duran-Aniotz, Victor Hugo Cornejo, Sandra Espinoza, Álvaro O Ardiles, Danilo B Medinas, Claudia Salazar, Andrew Foley, Ivana Gajardo, Peter Thielen, Takao Iwawaki, Wiep Scheper, Claudio Soto, Adrian G Palacios, Jeroen J M Hoozemans, Claudio Hetz
Altered proteostasis is a salient feature of Alzheimer's disease (AD), highlighting the occurrence of endoplasmic reticulum (ER) stress and abnormal protein aggregation. ER stress triggers the activation of the unfolded protein response (UPR), a signaling pathway that enforces adaptive programs to sustain proteostasis or eliminate terminally damaged cells. IRE1 is an ER-located kinase and endoribonuclease that operates as a major stress transducer, mediating both adaptive and proapoptotic programs under ER stress...
March 24, 2017: Acta Neuropathologica
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