25,915 research outputs found

    Bioprospecção de lípidos da alga vermelha Palmaria palmata como fonte de compostos bioactivos

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    Palmaria palmata is an edible red seaweed rich in the omega-3 eicosapentaenoic acid (EPA), mostly esterified with polar lipids (phospholipids and glycolipids). EPA is known for its positive effects in health, namely in the prevention of non-communicable diseases. However, the polar lipid content of P. palmata is still undervalued and poorly explored. Therefore, this work aimed to (a) bioprospect the polar lipids from P. palmata as a source of add-value compounds with potential health benefits, focusing on their anti-inflammatory and antioxidant properties, and (b) correlate the bioactive potential with the lipid composition, through an untargeted lipidomic approach, using HILIC-ESI-MS/MS to identify and characterize the polar lipid profile. The total lipid extract as well as the glycolipid- and phospholipid-enriched extracts were shown to be sources of compounds with antioxidant potential, scavenging the radicals ABTS‚óŹ+ and DPPH‚óŹ in chemico, and reducing the intracellular oxidative stress in vitro, in RAW macrophages treated with lipopolysaccharide (LPS). The lipid extracts also showed an anti-inflammatory potential, inhibiting the COX-2 activity in chemico, and reducing the production of nitric oxide in vitro and the transcription of pro-inflammatory genes such as Nos2, IL1b, IL6, Tnf e Ptgs2, triggered by LPS in RAW macrophages. These results show that both the phospholipid- and glycolipid-enriched extracts seem to have a relevant role in the bioactivities demonstrated by the total lipid extract. The characterization of the lipid extracts by HILIC-ESI-MS/MS highlighted that the biomass of the seaweed P. palmata farmed in an integrated multitrophic aquaculture (IMTA) is a source of compounds with high nutritional value ‚Äď namely omega-3 PUFAs ‚Äď and bioactive compounds, mainly EPA-rich polar lipids, thus contributing to its valorisation as a healthy and functional food. Future studies are necessary to evaluate the bioaccessibility and bioavailability of these bioactive lipids, in order to ensure that the properties observed in chemico and in vitro will show in their in vivo utilization.A Palmaria palmata √© uma macroalga vermelha comest√≠vel com um elevado teor de √°cido √≥mega-3 eicosapentan√≥ico (EPA) maioritariamente esterificado na forma de l√≠pidos polares (fosfol√≠pidos e glicol√≠pidos). O EPA √© conhecido pelos seus efeitos positivos na sa√ļde, nomeadamente na preven√ß√£o de doen√ßas n√£o comunic√°veis. No entanto, o conte√ļdo de l√≠pidos polares da P. palmata continua desvalorizado e pouco explorado. Assim, este trabalho teve como objetivos (a) a bioprospec√ß√£o dos l√≠pidos polares da P. palmata como fonte de compostos de valor acrescentado e potencialmente ben√©ficos para a sa√ļde, com √™nfase nas suas propriedades anti-inflamat√≥rias e antioxidantes, e (b) relacionar o potencial bioativo com a composi√ß√£o de l√≠pidos polares, atrav√©s duma abordagem lipid√≥mica, usando HILIC-ESI-MS/MS para a identifica√ß√£o e carateriza√ß√£o do perfil lip√≠dico. Quer o extrato lip√≠dico total quer as fra√ß√Ķes enriquecidas em fosfol√≠pidos e glicol√≠pidos revelaram-se fontes de compostos com potencial antioxidante, reduzindo os radicais ABTS‚óŹ+ e DPPH‚óŹ in chemico e diminuindo o stress oxidativo intracelular in vitro, em macr√≥fagos RAW tratados com lipopolissacar√≠deo (LPS). Os extratos lip√≠dicos tamb√©m demonstraram ter potencial anti-inflamat√≥rio, inibindo a atividade da COX-2 in chemico, e diminuindo a produ√ß√£o de √≥xido n√≠trico in vitro e a transcri√ß√£o de genes pro-inflamat√≥rios tais como Nos2, IL1b, IL6, Tnf e Ptgs2, desencadeada por LPS em macr√≥fagos RAW. Estes resultados mostram que as fra√ß√Ķes de fosfol√≠pidos e glicol√≠pidos parecem ter um papel relevante nas bioatividades demonstradas pelo extrato lip√≠dico total. A caracteriza√ß√£o dos extratos lip√≠dicos por HILIC-ESI-MS/MS evidenciou a sua riqueza em esp√©cies moleculares contendo EPA, tendo este perfil sido conservado ap√≥s o fracionamento em fosfol√≠pidos e glicolipidos. Este trabalho demonstrou que a biomassa da macroalga P. palmata cultivada em aquacultura multitr√≥fica integrada (IMTA) √© uma fonte de compostos de elevado valor nutricional - nomeadamente √≥mega-3 PUFAs - e de compostos bioactivos, nomeadamente l√≠pidos polares ricos em EPA, contribuindo assim para a sua valoriza√ß√£o enquanto alimento saud√°vel e funcional. Estudos futuros s√£o necess√°rios para avaliar a bioacessibilidade e biodisponibilidade destes l√≠pidos bioativos, de forma a garantir que as propriedades observadas in chemico e in vitro tenham reflexo na sua utiliza√ß√£o in vivo.Mestrado em Bioqu√≠mic

    Short exposure to photo-oxidative damage triggers molecular signals indicative of early retinal degeneration

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    IntroductionAge-related macular degeneration (AMD) is the leading cause of blindness in the developed world, currently affecting over 350 billion people globally. For the most prevalent late-stage form of this disease, atrophic AMD, there are no available prevention strategies or treatments, in part due to inherent difficulties in early-stage diagnosis. Photo-oxidative damage is a well-established model for studying inflammatory and cell death features that occur in late-stage atrophic AMD, however to date has not been investigated as a potential model for studying early features of disease onset. Therefore, in this study we aimed to determine if short exposure to photo-oxidative damage could be used to induce early retinal molecular changes and advance this as a potential model for studying early-stage AMD.MethodsC57BL/6J mice were exposed to 1, 3, 6, 12, or 24h photo-oxidative damage (PD) using 100k lux bright white light. Mice were compared to dim-reared (DR) healthy controls as well as mice which had undergone long periods of photo-oxidative damage (3d and 5d-PD) as known timepoints for inducing late-stage retinal degeneration pathologies. Cell death and retinal inflammation were measured using immunohistochemistry and qRT-PCR. To identify retinal molecular changes, retinal lysates were sent for RNA sequencing, following which bioinformatics analyses including differential expression and pathway analyses were performed. Finally, to investigate modulations in gene regulation as a consequence of degeneration, microRNA (miRNA) expression patterns were quantified using qRT-PCR and visualized using in situ hybridization.ResultsShort exposure to photo-oxidative damage (1-24h-PD) induced early molecular changes in the retina, with progressive downregulation of homeostatic pathways including metabolism, transport and phototransduction observed across this time-course. Inflammatory pathway upregulation was observed from 3h-PD, preceding observable levels of microglia/macrophage activation which was noted from 6h-PD, as well as significant photoreceptor row loss from 24h-PD. Further rapid and dynamic movement of inflammatory regulator miRNA, miR-124-3p and miR-155-5p, was visualized in the retina in response to degeneration.ConclusionThese results support the use of short exposure to photo-oxidative damage as a model of early AMD and suggest that early inflammatory changes in the retina may contribute to pathological features of AMD progression including immune cell activation and photoreceptor cell death. We suggest that early intervention of these inflammatory pathways by targeting miRNA such as miR-124-3p and miR-155-5p or their target genes may prevent progression into late-stage pathology

    Pollution-induced community tolerance in freshwater biofilms ‚Äď from molecular mechanisms to loss of community functions

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    Exposure to herbicides poses a threat to aquatic biofilms by affecting their community structure, physiology and function. These changes render biofilms to become more tolerant, but on the downside community tolerance has ecologic costs. A concept that addresses induced community tolerance to a pollutant (PICT) was introduced by Blanck and W√§ngberg (1988). The basic principle of the concept is that microbial communities undergo pollution-induced succession when exposed to a pollutant over a long period of time, which changes communities structurally and functionally and enhancing tolerance to the pollutant exposure. However, the mechanisms of tolerance and the ecologic consequences were hardly studied up to date. This thesis addresses the structural and functional changes in biofilm communities and applies modern molecular methods to unravel molecular tolerance mechanisms. Two different freshwater biofilm communities were cultivated for a period of five weeks, with one of the communities being contaminated with 4 őľg L-1 diuron. Subsequently, the communities were characterized for structural and functional differences, especially focusing on their crucial role of photosynthesis. The community structure of the autotrophs was assessed using HPLC-based pigment analysis and their functional alterations were investigated using Imaging-PAM fluorometry to study photosynthesis and community oxygen profiling to determine net primary production. Then, the molecular fingerprints of the communities were measured with meta-transcriptomics (RNA-Seq) and GC-based community metabolomics approaches and analyzed with respect to changes in their molecular functions. The communities were acute exposed to diuron for one hour in a dose-response design, to reveal a potential PICT and uncover related adaptation to diuron exposure. The combination of apical and molecular methods in a dose-response design enabled the linkage of functional effects of diuron exposure and underlying molecular mechanisms based on a sensitivity analysis. Chronic exposure to diuron impaired freshwater biofilms in their biomass accrual. The contaminated communities particularly lost autotrophic biomass, reflected by the decrease in specific chlorophyll a content. This loss was associated with a change in the molecular fingerprint of the communities, which substantiates structural and physiological changes. The decline in autotrophic biomass could be due to a primary loss of sensitive autotrophic organisms caused by the selection of better adapted species in the course of chronic exposure. Related to this hypothesis, an increase in diuron tolerance has been detected in the contaminated communities and molecular mechanisms facilitating tolerance have been found. It was shown that genes of the photosystem, reductive-pentose phosphate cycle and arginine metabolism were differentially expressed among the communities and that an increased amount of potential antioxidant degradation products was found in the contaminated communities. This led to the hypothesis that contaminated communities may have adapted to oxidative stress, making them less sensitive to diuron exposure. Moreover, the photosynthetic light harvesting complex was altered and the photoprotective xanthophyll cycle was increased in the contaminated communities. Despite these adaptation strategies, the loss of autotrophic biomass has been shown to impair primary production. This impairment persisted even under repeated short-term exposure, so that the tolerance mechanisms cannot safeguard primary production as a key function in aquatic systems.:1. The effect of chemicals on organisms and their functions .............................. 1 1.1 Welcome to the anthropocene .......................................................................... 1 1.2 From cellular stress responses to ecosystem resilience ................................... 3 1.2.1 The individual pursuit for homeostasis ....................................................... 3 1.2.2 Stability from diversity ................................................................................. 5 1.3 Community ecotoxicology - a step forward in monitoring the effects of chemical pollution? ................................................................................................................. 6 1.4 Functional ecotoxicological assessment of microbial communities ................... 9 1.5 Molecular tools ‚Äď the key to a mechanistic understanding of stressor effects from a functional perspective in microbial communities? ...................................... 12 2. Aims and Hypothesis ......................................................................................... 14 2.1 Research question .......................................................................................... 14 2.2 Hypothesis and outline .................................................................................... 15 2.3 Experimental approach & concept .................................................................. 16 2.3.1 Aquatic freshwater biofilms as model community ..................................... 16 2.3.2 Diuron as model herbicide ........................................................................ 17 2.3.3 Experimental design ................................................................................. 18 3. Structural and physiological changes in microbial communities after chronic exposure - PICT and altered functional capacity ................................................. 21 3.1 Introduction ..................................................................................................... 21 3.2 Methods .......................................................................................................... 23 3.2.1 Biofilm cultivation ...................................................................................... 23 3.2.2 Dry weight and autotrophic index ............................................................. 23 3.2.4 Pigment analysis of periphyton ................................................................. 23 3.2.4.1 In-vivo pigment analysis for community characterization ....................... 24 3.2.4.2 In-vivo pigment analysis based on Imaging-PAM fluorometry ............... 24 3.2.4.3 In-vivo pigment fluorescence for tolerance detection ............................. 26 3.2.4.4 Ex-vivo pigment analysis by high-pressure liquid-chromatography ....... 27 3.2.5 Community oxygen metabolism measurements ....................................... 28 3.3 Results and discussion ................................................................................... 29 3.3.1 Comparison of the structural community parameters ............................... 29 3.3.2 Photosynthetic activity and primary production of the communities after selection phase ................................................................................................. 33 3.3.3 Acquisition of photosynthetic tolerance .................................................... 34 3.3.4 Primary production at exposure conditions ............................................... 36 3.3.5 Tolerance detection in primary production ................................................ 37 3.4 Summary and Conclusion ........................................................................... 40 4. Community gene expression analysis by meta-transcriptomics ................... 41 4.1 Introduction to meta-transcriptomics ............................................................... 41 4.2. Methods ......................................................................................................... 43 4.2.1 Sampling and RNA extraction................................................................... 43 4.2.2 RNA sequencing analysis ......................................................................... 44 4.2.3 Data assembly and processing................................................................. 45 4.2.4 Prioritization of contigs and annotation ..................................................... 47 4.2.5 Sensitivity analysis of biological processes .............................................. 48 4.3 Results and discussion ................................................................................... 48 4.3.1 Characterization of the meta-transcriptomic fingerprints .......................... 49 4.3.2 Insights into community stress response mechanisms using trend analysis (DRomic‚Äôs) ......................................................................................................... 51 4.3.3 Response pattern in the isoform PS genes .............................................. 63 4.5 Summary and conclusion ................................................................................ 65 5. Community metabolome analysis ..................................................................... 66 5.1 Introduction to community metabolomics ........................................................ 66 5.2 Methods .......................................................................................................... 68 5.2.1 Sampling, metabolite extraction and derivatisation................................... 68 5.2.2 GC-TOF-MS analysis ............................................................................... 69 5.2.3 Data processing and statistical analysis ................................................... 69 5.3 Results and discussion ................................................................................... 70 5.3.1 Characterization of the metabolic fingerprints .......................................... 70 5.3.2 Difference in the metabolic fingerprints .................................................... 71 5.3.3 Differential metabolic responses of the communities to short-term exposure of diuron ............................................................................................................ 73 5.4 Summary and conclusion ................................................................................ 78 6. Synthesis ............................................................................................................. 79 6.1 Approaches and challenges for linking molecular data to functional measurements ...................................................................................................... 79 6.2 Methods .......................................................................................................... 83 6.2.1 Summary on the data ............................................................................... 83 6.2.2 Aggregation of molecular data to index values (TELI and MELI) .............. 83 6.2.3 Functional annotation of contigs and metabolites using KEGG ................ 83 6.3 Results and discussion ................................................................................... 85 6.3.1 Results of aggregation techniques ........................................................... 85 6.3.2 Sensitivity analysis of the different molecular approaches and endpoints 86 6.3.3 Mechanistic view of the molecular stress responses based on KEGG functions ............................................................................................................ 89 6.4 Consolidation of the results ‚Äď holistic interpretation and discussion ............... 93 6.4.1 Adaptation to chronic diuron exposure - from molecular changes to community effects.............................................................................................. 93 6.4.2 Assessment of the ecological costs of Pollution-induced community tolerance based on primary production ............................................................. 94 6.5 Outlook ............................................................................................................ 9

    Selenium nanoparticles modulate histone methylation via lysine methyltransferase activity and S-adenosylhomocysteine depletion

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    At physiological levels, the trace element selenium plays a key role in redox reactions through the incorporation of selenocysteine in antioxidant enzymes. Selenium has also been evaluated as a potential anti-cancer agent, where selenium nanoparticles have proven effective, and are well tolerated in vivo at doses that are toxic as soluble Se. The use of such nanoparticles, coated with either serum albumin or the naturally occurring alkaline polysaccharide chitosan, also serves to enhance biocompatibility and bioavailability. Here we demonstrate a novel role for selenium in regulating histone methylation in ovarian cancer cell models treated with inorganic selenium nanoparticles coated with serum albumin or chitosan. As well as inducing thioredoxin reductase expression, ROS activity and cancer cell cytotoxicity, coated nanoparticles caused significant increases in histone methylation. Specifically, selenium nanoparticles triggered an increase in the methylation of histone 3 at lysines K9 and K27, histone marks involved in both the activation and repression of gene expression, thus suggesting a fundamental role for selenium in these epigenetic processes. This direct function was confirmed using chemical inhibitors of the histone lysine methyltransferases EZH2 (H3K27) and G9a/EHMT2 (H3K9), both of which blocked the effect of selenium on histone methylation. This novel role for selenium supports a distinct function in histone methylation that occurs due to a decrease in S-adenosylhomocysteine, an endogenous inhibitor of lysine methyltransferases, the metabolic product of methyl-group transfer from S-adenosylmethionine in the one-carbon metabolism pathway. These observations provide important new insights into the action of selenium nanoparticles. It is now important to consider both the classic antioxidant and novel histone methylation effects of this key redox element in its development in cancer therapy and other applications

    Diagnosis and Treatment in Asthma and Allergic Rhinitis: Past, Present, and Future

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    Respiratory diseases are pathological conditions that affect airways, hampering breathing and causing high mortality. In particular, asthma and allergic rhinitis (AR) are two of the most common airway diseases that affect millions of people and have a high prevalence in childhood and adulthood. Asthma is a heterogeneous chronic inflammatory disease characterized by wheezing, chest tightness, shortness of breath, and cough. AR occurs with rhinorrhea, nasal congestion, and sneezing. Indeed, these pathologies share common physiopathological mechanisms such as airway hyperresponsiveness and similar immunopathology such as tissue eosinophilia and T-helper type 2 inflammation. Moreover, AR can be an important risk factor for suffering asthma. Thus, early diagnosis and effective treatment are crucial to improving the health and quality of life of these patients. Classical drugs such as corticosteroids have been used; however, in the last decades, efforts to improve treatments have increased, focusing on biological agents and specific allergen immunotherapy development. Moreover, more precise diagnostic tools have been elaborated, besides classical methods (medical history, physical examination, and pulmonary function tests), such as basophil activation test, and specific cellular and molecular biomarkers (microRNAs, sputum/blood eosinophils, IgE serum, and periostin levels). Therefore, in this review, we compile all these important issues for managing asthma and AR.Espada-S√°nchez M, S√°enz de Santa Mar√≠a R, Mart√≠n-Astorga MdC, Lebr√≥n-Mart√≠n C, Delgado MJ, Eguiluz-Gracia I, Rond√≥n C, Mayorga C, Torres MJ, Aranda CJ, Ca√Īas JA. Diagnosis and Treatment in Asthma and Allergic Rhinitis: Past, Present, and Future. Applied Sciences. 2023; 13(3):1273. https://doi.org/10.3390/app1303127

    Role of NLRP3 in the pathogenesis and treatment of gout arthritis

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    Gout arthritis (GA) is a common and curable type of inflammatory arthritis that has been attributed to a combination of genetic, environmental and metabolic factors. Chronic deposition of monosodium urate (MSU) crystals in articular and periarticular spaces as well as subsequent activation of innate immune system in the condition of persistent hyperuricemia are the core mechanisms of GA. As is well known, drugs for GA therapy primarily consists of rapidly acting anti-inflammatory agents and life-long uric acid lowering agents, and their therapeutic outcomes are far from satisfactory. Although MSU crystals in articular cartilage detected by arthrosonography or in synovial fluid found by polarization microscopy are conclusive proofs for GA, the exact molecular mechanism of NLRP3 inflammasome activation in the course of GA still remains mysterious, severely restricting the early diagnosis and therapy of GA. On the one hand, the activation of Nod-like receptor family, pyrin domain containing 3 (NLRP3) inflammasome requires nuclear factor kappa B (NF-őļB)-dependent transcriptional enhancement of NLRP3, precursor (pro)-caspase-1 and pro-IL-1ő≤, as well as the assembly of NLRP3 inflammasome complex and sustained release of inflammatory mediators and cytokines such as IL-1ő≤, IL-18 and caspase-1. On the other hand, NLRP3 inflammasome activated by MSU crystals is particularly relevant to the initiation and progression of GA, and thus may represent a prospective diagnostic biomarker and therapeutic target. As a result, pharmacological inhibition of the assembly and activation of NLRP3 inflammasome may also be a promising avenue for GA therapy. Herein, we first introduced the functional role of NLRP3 inflammasome activation and relevant biological mechanisms in GA based on currently available evidence. Then, we systematically reviewed therapeutic strategies for targeting NLRP3 by potentially effective agents such as natural products, novel compounds and noncoding RNAs (ncRNAs) in the treatment of MSU-induced GA mouse models. In conclusion, our present review may have significant implications for the pathogenesis, diagnosis and therapy of GA

    Microbial Mitigation of Drought Stress in Plants: Adaptations to Climate Change

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    The global temperature is constantly increasing due to the phenomenon of climate change. Plants have developed various mechanisms to defend themselves against environmental stresses including drought stress. Apart from indigenous biochemical, physiological, and molecular mechanisms of adaptation to stress, the plant-associated microbes may also play a crucial role in plant drought tolerance. The endophytic and rhizospheric microbes perform various functions and produce different enzymes and compounds that play an important role in plants’ adaptation to various environmental stresses including drought stress. Some of the key mechanisms include production of growth hormones, siderophores, organic acids, induction of the ROS scavenging system, phosphate solubilization, and nitrogen fixation. However, the production of ACC deaminase in the plant-associated microbes has vital roles in reduction of ethylene levels under drought stress, resulting in improved plant growth and stress tolerance. Owing to the complex nature of drought tolerance, a multi-pronged approach would have to be adapted to further enhance the microbial-mediated drought tolerance in plants

    Anu√°rio cient√≠fico da Escola Superior de Tecnologia da Sa√ļde de Lisboa - 2021

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    √Č com grande prazer que apresentamos a mais recente edi√ß√£o (a 11.¬™) do Anu√°rio Cient√≠fico da Escola Superior de Tecnologia da Sa√ļde de Lisboa. Como institui√ß√£o de ensino superior, temos o compromisso de promover e incentivar a pesquisa cient√≠fica em todas as √°reas do conhecimento que contemplam a nossa miss√£o. Esta publica√ß√£o tem como objetivo divulgar toda a produ√ß√£o cient√≠fica desenvolvida pelos Professores, Investigadores, Estudantes e Pessoal n√£o Docente da ESTeSL durante 2021. Este Anu√°rio √©, assim, o reflexo do trabalho √°rduo e dedicado da nossa comunidade, que se empenhou na produ√ß√£o de conte√ļdo cient√≠fico de elevada qualidade e partilhada com a Sociedade na forma de livros, cap√≠tulos de livros, artigos publicados em revistas nacionais e internacionais, resumos de comunica√ß√Ķes orais e p√≥steres, bem como resultado dos trabalhos de 1¬ļ e 2¬ļ ciclo. Com isto, o conte√ļdo desta publica√ß√£o abrange uma ampla variedade de t√≥picos, desde temas mais fundamentais at√© estudos de aplica√ß√£o pr√°tica em contextos espec√≠ficos de Sa√ļde, refletindo desta forma a pluralidade e diversidade de √°reas que definem, e tornam √ļnica, a ESTeSL. Acreditamos que a investiga√ß√£o e pesquisa cient√≠fica √© um eixo fundamental para o desenvolvimento da sociedade e √© por isso que incentivamos os nossos estudantes a envolverem-se em atividades de pesquisa e pr√°tica baseada na evid√™ncia desde o in√≠cio dos seus estudos na ESTeSL. Esta publica√ß√£o √© um exemplo do sucesso desses esfor√ßos, sendo a maior de sempre, o que faz com que estejamos muito orgulhosos em partilhar os resultados e descobertas dos nossos investigadores com a comunidade cient√≠fica e o p√ļblico em geral. Esperamos que este Anu√°rio inspire e motive outros estudantes, profissionais de sa√ļde, professores e outros colaboradores a continuarem a explorar novas ideias e contribuir para o avan√ßo da ci√™ncia e da tecnologia no corpo de conhecimento pr√≥prio das √°reas que comp√Ķe a ESTeSL. Agradecemos a todos os envolvidos na produ√ß√£o deste anu√°rio e desejamos uma leitura inspiradora e agrad√°vel.info:eu-repo/semantics/publishedVersio

    Protein quality control and aggregation in the endoplasmic reticulum: From basic to bedside

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    Endoplasmic reticulum (ER) is the largest membrane-bound compartment in all cells and functions as a key regulator in protein biosynthesis, lipid metabolism, and calcium balance. Mammalian endoplasmic reticulum has evolved with an orchestrated protein quality control system to handle defective proteins and ensure endoplasmic reticulum homeostasis. Nevertheless, the accumulation and aggregation of misfolded proteins in the endoplasmic reticulum may occur during pathological conditions. The inability of endoplasmic reticulum quality control system to clear faulty proteins and aggregates from the endoplasmic reticulum results in the development of many human disorders. The efforts to comprehensively understand endoplasmic reticulum quality control network and protein aggregation will benefit the diagnostics and therapeutics of endoplasmic reticulum storage diseases. Herein, we overview recent advances in mammalian endoplasmic reticulum protein quality control system, describe protein phase transition model, and summarize the approaches to monitor protein aggregation. Moreover, we discuss the therapeutic applications of enhancing endoplasmic reticulum protein quality control pathways in endoplasmic reticulum storage diseases
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