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https://www.readbyqxmd.com/read/28819167/artificial-dnaj-protein-for-protein-production-and-conformational-diseases
#1
Akinori Hishiya, Keizo Koya
For secreted proteins, proper protein folding is essential not only for biological function but also for secretion itself. Proteins with folding problems are trapped in the endoplasmic reticulum (ER) and are eventually degraded in the cytoplasm. In this study, we exploited co-expression of an artificial fusion protein, based on the sequence of a DnaJ protein, which could interact as co-chaperones in the Hsp70-based protein-folding system, with target recombinant secreted proteins to enhance their production and secretion...
August 17, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28814853/improving-adherence-to-alpha-1-antitrypsin-deficiency-screening-guidelines-using-the-pulmonary-function-laboratory
#2
Landy V Luna Diaz, Isabella Iupe, Bruno Zavala, Kira C Balestrini, Andrea Guerrero, Gregory Holt, Rafael Calderon-Candelario, Mehdi Mirsaeidi, Michael Campos
No abstract text is available yet for this article.
2017: International Journal of Chronic Obstructive Pulmonary Disease
https://www.readbyqxmd.com/read/28814511/targeting-phospholipase-d4-attenuates-kidney-fibrosis
#3
Priyanka Trivedi, Ramya K Kumar, Ashwin Iyer, Sarah Boswell, Casimiro Gerarduzzi, Vivekkumar P Dadhania, Zach Herbert, Nikita Joshi, James P Luyendyk, Benjamin D Humphreys, Vishal S Vaidya
Phospholipase D4 (PLD4), a single-pass transmembrane glycoprotein, is among the most highly upregulated genes in murine kidneys subjected to chronic progressive fibrosis, but the function of PLD4 in this process is unknown. Here, we found PLD4 to be overexpressed in the proximal and distal tubular epithelial cells of murine and human kidneys after fibrosis. Genetic silencing of PLD4, either globally or conditionally in proximal tubular epithelial cells, protected mice from the development of fibrosis. Mechanistically, global knockout of PLD4 modulated innate and adaptive immune responses and attenuated the upregulation of the TGF-β signaling pathway and α1-antitrypsin protein (a serine protease inhibitor) expression and downregulation of neutrophil elastase (NE) expression induced by obstructive injury...
August 16, 2017: Journal of the American Society of Nephrology: JASN
https://www.readbyqxmd.com/read/28794206/lipid-based-nutrient-supplements-during-pregnancy-and-lactation-did-not-affect-human-milk-oligosaccharides-and-bioactive-proteins-in-a-randomized-trial
#4
Josh M Jorgensen, Charles Arnold, Per Ashorn, Ulla Ashorn, David Chaima, Yin Bun Cheung, Jasmine Cc Davis, Yue-Mei Fan, Elisha Goonatilleke, Emma Kortekangas, Chiza Kumwenda, Carlito B Lebrilla, Kenneth Maleta, Sarah M Totten, Lauren D Wu, Kathryn G Dewey
Background: Human milk oligosaccharides (HMOs) and bioactive proteins are beneficial to infant health. Recent evidence suggests that maternal nutrition may affect the amount of HMOs and proteins in breast milk; however, the effect of nutrient supplementation on HMOs and bioactive proteins has not yet been well studied.Objective: We aimed to determine whether lipid-based nutrient supplements (LNSs) affect milk bioactive protein and HMO concentrations at 6 mo postpartum in women in rural Malawi. These are secondary outcomes of a previously published randomized controlled trial...
August 9, 2017: Journal of Nutrition
https://www.readbyqxmd.com/read/28793328/liver-tissue-fragments-obtained-from-males-are-the-most-promising-source-of-human-hepatocytes-for-cell-based-therapies-flow-cytometric-analysis-of-albumin-expression
#5
Karolina Ewa Zakrzewska, Anna Samluk, Agnieszka Wencel, Krzysztof Dudek, Dorota Genowefa Pijanowska, Krzysztof Dariusz Pluta
Cell-based therapies that could provide an alternative treatment for the end-stage liver disease require an adequate source of functional hepatocytes. There is little scientific evidence for the influence of patient's age, sex, and chemotherapy on the cell isolation efficiency and metabolic activity of the harvested hepatocytes. The purpose of this study was to investigate whether hepatocytes derived from different sources display differential viability and biosynthetic capacity. Liver cells were isolated from 41 different human tissue specimens...
2017: PloS One
https://www.readbyqxmd.com/read/28793213/critical-influence-of-cosolutes-and-surfaces-on-the-assembly-of-serpin-derived-amyloid-fibrils
#6
Michael W Risør, Dennis W Juhl, Morten Bjerring, Joachim Mathiesen, Jan J Enghild, Niels C Nielsen, Daniel E Otzen
Many proteins and peptides self-associate into highly ordered and structurally similar amyloid cross-β aggregates. This fibrillation is critically dependent on properties of the protein and the surrounding environment that alter kinetic and thermodynamic equilibria. Here, we report on dominating surface and solution effects on the fibrillogenic behavior and amyloid assembly of the C-36 peptide, a circulating bioactive peptide from the α1-antitrypsin serine protease inhibitor. C-36 converts from an unstructured peptide to mature amyloid twisted-ribbon fibrils over a few hours when incubated on polystyrene plates under physiological conditions through a pathway dominated by surface-enhanced nucleation...
August 8, 2017: Biophysical Journal
https://www.readbyqxmd.com/read/28783388/supraclavicular-aneurysm-as-a-presentation-of-alpha-1-antitrypsin-deficiency
#7
Pablo Ruiz-Sada, Mikel Eskalante-Boleas, Iker Garay-Hidalgo, Lara Palacios-García
No abstract text is available yet for this article.
August 2, 2017: British Journal of Hospital Medicine
https://www.readbyqxmd.com/read/28769553/clinical-utility-of-alpha-1-proteinase-inhibitor-in-the-management-of-adult-patients-with-severe-alpha-1-antitrypsin-deficiency-a-review-of-the-current-literature
#8
REVIEW
David G Parr, Beatriz Lara
Alpha-1 antitrypsin (AAT) functions primarily to inhibit neutrophil elastase, and its deficiency predisposes individuals to the development of chronic obstructive pulmonary disease (COPD). The putative protective serum concentration is generally considered to be above a threshold of 11 μM/L, and therapeutic augmentation of AAT above this value is believed to retard the progression of emphysema. Several AAT preparations, all derived from human donor plasma, have been commercialized since approval by the US Food and Drug Administration (FDA) in 1987...
2017: Drug Design, Development and Therapy
https://www.readbyqxmd.com/read/28762562/co-expression-of-alpha-1-antitrypsin-with-cytoplasmic-domain-of-v-snare-in-pichia-pastoris-preserving-biological-activity-of-alpha-1-antitrypsin
#9
Maryam Khatami, Seyed Nezamedin Hosseini, Sadegh Hasannia
Alpha-1-antitrypsin (A1AT) is a major serum protein in human with protease inhibitory activity. Because of its extensive application in medicine, recombinant DNA technology has been considered for its production. The current study is going to examine co-expression of A1AT, and soluble domain of v-SNARE in Pichia pastoris, which can prevent the secretion of A1AT after passing the secretory pathway thoroughly. This was done mainly to preserve the biological activity of A1AT which in the secretory mode might be impaired in the fermentation and early clarification conditions...
August 1, 2017: Biotechnology and Applied Biochemistry
https://www.readbyqxmd.com/read/28761367/protein-losing-enteropathy-comprehensive-review-of-the-mechanistic-association-with-clinical-and-subclinical-disease-states
#10
REVIEW
David G Levitt, Michael D Levitt
Protein losing enteropathy (PLE) has been associated with more than 60 different conditions, including nearly all gastrointestinal diseases (Crohn's disease, celiac, Whipple's, intestinal infections, and so on) and a large number of non-gut conditions (cardiac and liver disease, lupus, sarcoidosis, and so on). This review presents the first attempt to quantitatively understand the magnitude of the PLE in relation to the associated pathology for three different disease categories: 1) increased lymphatic pressure (e...
2017: Clinical and Experimental Gastroenterology
https://www.readbyqxmd.com/read/28759613/acute-phase-proteins-as-prospective-risk-markers-for-arterial-stiffness-the-malm%C3%A3-diet-and-cancer-cohort
#11
Iram Faqir Muhammad, Yan Borné, Gerd Östling, Cecilia Kennbäck, Mikael Gottsäter, Margaretha Persson, Peter M Nilsson, Gunnar Engström
BACKGROUND AND OBJECTIVES: Arterial stiffness plays a significant role in the development and progression of adverse cardiovascular events and all-cause mortality. This observational study aims to explore the relationship between six acute phase proteins namely, ceruloplasmin, alpha-1-antitrypsin, orosomucoid, haptoglobin, complement C3 and C-reactive protein (CRP), and carotid-femoral pulse wave velocity (c-f PWV) in a population-based cohort, and to also explore the effect of low-grade inflammation on the relationship between diabetes and c-f PWV...
2017: PloS One
https://www.readbyqxmd.com/read/28759053/the-eukaryotic-gut-virome-in-hematopoietic-stem-cell-transplantation-new-clues-in-enteric-graft-versus-host-disease
#12
Jérôme Legoff, Matthieu Resche-Rigon, Jerome Bouquet, Marie Robin, Samia N Naccache, Séverine Mercier-Delarue, Scot Federman, Erik Samayoa, Clotilde Rousseau, Prescillia Piron, Nathalie Kapel, François Simon, Gérard Socié, Charles Y Chiu
Much attention has been focused on the role of the bacterial microbiome in human health, but the virome is understudied. Although previously investigated in individuals with inflammatory bowel diseases or solid-organ transplants, virome dynamics in allogeneic hematopoietic stem cell transplantation (HSCT) and enteric graft-versus-host disease (GVHD) remain unexplored. Here we characterize the longitudinal gut virome in 44 recipients of HSCT using metagenomics. A viral 'bloom' was identified, and significant increases were demonstrated in the overall proportion of vertebrate viral sequences following transplantation (P = 0...
July 31, 2017: Nature Medicine
https://www.readbyqxmd.com/read/28753703/-a-rare-cause-of-a-spontaneous-pneumothorax
#13
M Lepiorz, C Großer, H-S Hofmann, M Pfeifer
A young patient presented himself to the emergency department with sudden-onset, breathing-dependent right-sided thoracic pain. The auscultation revealed diminished breath sounds on the right. The radiograph showed a pneumothorax which was immediately dealt with chest tube drainage. The CT scan of the thorax showed minuscule subpleural bullae. Video-assisted thoracoscopic surgery (VATS) was performed due to persistent fistulae formation through the drain. The subpleural, bullous and emphysematous changes were histologically confirmed...
July 28, 2017: Pneumologie
https://www.readbyqxmd.com/read/28752467/therapeutics-gene-therapy-for-alpha-1-antitrypsin-deficiency
#14
Alisha M Gruntman, Terence R Flotte
This review seeks to give an overview of alpha-1 antitrypsin deficiency, including the different disease phenotypes that it encompasses. We then describe the different therapeutic endeavors that have been undertaken to address these different phenotypes. Lastly we discuss future potential therapeutics, such as genome editing, and how they may play a role in treating alpha-1 antitrypsin deficiency.
2017: Methods in Molecular Biology
https://www.readbyqxmd.com/read/28752466/therapeutic-options-in-alpha-1-antitrypsin-deficiency-liver-transplantation
#15
Nedim Hadzic
Alpha-1 antitrypsin deficiency is the commonest genetic condition leading to liver transplantation in childhood. It remains unclear why only a minority of individuals carrying homozygous PiZ phenotype has liver disease, but also why of those only about a quarter develops end stage liver disease, requiring liver transplantation. This intervention has now become routine worldwide with 1-year patient survival rates well above 90%. As for all autosomal recessive conditions liver donation from anonymous cadaveric sources is preferred to living related parental donors, due to their presumed heterozygous state...
2017: Methods in Molecular Biology
https://www.readbyqxmd.com/read/28752465/therapeutics-alpha-1-antitrypsin-augmentation-therapy
#16
Michael Campos, Jorge Lascano
Subjects with alpha-1 antitrypsin deficiency who develop pulmonary disease are managed following general treatment guidelines, including disease management interventions. In addition, administration of intravenous infusions of alpha-1 proteinase inhibitor (augmentation therapy) at regular schedules is a specific therapy for individuals with AATD with pulmonary involvement.This chapter summarizes the manufacturing differences of commercially available formulations and the available evidence of the effects of augmentation therapy...
2017: Methods in Molecular Biology
https://www.readbyqxmd.com/read/28752464/electrophoresis-and-fret-based-measures-of-serpin-polymerization
#17
Sarah V Faull, Anwen E Brown, Imran Haq, James A Irving
Many serpinopathies, including alpha-1 antitrypsin (A1AT) deficiency, are associated with the formation of unbranched polymer chains of mutant serpins. In vivo, this deficiency is the result of mutations that cause kinetic or thermodynamic destabilization of the molecule. However, polymerization can also be induced in vitro from mutant or wild-type serpins under destabilizing conditions. The characteristics of the resulting polymers are dependent upon induction conditions. Due to their relationship to disease, serpin polymers, mainly those formed from A1AT, have been widely studied...
2017: Methods in Molecular Biology
https://www.readbyqxmd.com/read/28752463/semiquantitation-of-monomer-and-polymer-alpha-1-antitrypsin-by-centrifugal-separation-and-assay-by-western-blot-of-soluble-and-insoluble-components
#18
Keith S Blomenkamp, Jeffrey H Teckman
Alpha-1 antitrypsin (a1AT) deficiency, in its classical form, is an autosomal recessive disease associated with an increased risk of liver disease in adults and children, and with lung disease in adults. The vast majority of liver disease is associated with homozygosity for the Z mutant allele, also called PiZZ. This homozygous allele synthesizes large quantities of a1AT mutant Z protein in the liver, but the mutant protein also folds improperly during biogenesis. As a result, approximately 85% of the molecules are retained within the hepatocytes instead of being appropriately secreted...
2017: Methods in Molecular Biology
https://www.readbyqxmd.com/read/28752462/quantification-of-z-aat-by-a-z-specific-sandwich-elisa
#19
Florie Borel, Qiushi Tang, Christian Mueller
This protocol describes an enzyme-linked immunosorbent assay (ELISA) to specifically detect Z-alpha-1 antitrypsin (AAT), the most common protein variant associated with alpha-1 antitrypsin deficiency. This "sandwich" ELISA relies on an anti-Z-AAT specific capture antibody and a HRP-conjugated anti-AAT detection antibody. This method would be of interest to identify and quantify Z-AAT in a variety of samples such as cell culture medium, cell or tissue lysate, animal or patient serum. Because this method is specific and sensitive, it would be particularly valuable for detection of Z-AAT in the presence of background M-AAT, for instance when quantifying silencing of Z-AAT in patients undergoing M-AAT augmentation therapy...
2017: Methods in Molecular Biology
https://www.readbyqxmd.com/read/28752461/quantification-of-murine-aat-by-direct-elisa
#20
Andrew Cox, Christian Mueller
This methods chapter elaborates on how a direct enzyme-linked immunosorbent assay (ELISA) is used to specifically detect and quantify murine alpha-1 antitrypsin (AAT). As a direct ELISA, it lacks some sensitivity as compared to the "sandwich" ELISA method; however, it does reliably differentiate between samples with varying amounts of the mouse AAT protein. This protocol relies on the principle of adsorption to coat each well with sera proteins, whereas detection occurs specifically using a two-step antibody combination...
2017: Methods in Molecular Biology
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