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Antitrypsin

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https://www.readbyqxmd.com/read/28217691/%C3%AE-1-antitrypsin-deficiency-a-misfolded-secretory-protein-variant-with-unique-effects-on-the-endoplasmic-reticulum
#1
David H Perlmutter
In the classical form of α1-antitrypsin deficiency (ATD) a point mutation leads to accumulation of a misfolded secretory glycoprotein in the endoplasmic reticulum (ER) of liver cells and so ATD has come to be considered a prototypical ER storage disease. It is associated with two major types of clinical disorders, chronic obstructive pulmonary disease (COPD) by loss-of-function mechanisms and hepatic cirrhosis and carcinogenesis by gain-of-function mechanisms. The lung disease predominantly results from proteolytic damage to the pulmonary connective tissue matrix because of reduced levels of protease inhibitor activity of α1-anitrypsin (AT) in the circulating blood and body fluids...
September 2016: Endoplasmic Reticulum Stress in Diseases
https://www.readbyqxmd.com/read/28217373/update-on-clinical-and-research-application-of-fecal-biomarkers-for-gastrointestinal-diseases
#2
REVIEW
Imran Siddiqui, Hafsa Majid, Shahab Abid
Gastrointestinal (GI) diseases comprise a large spectrum of clinical conditions ranging from indigestion to inflammatory bowel diseases (IBDs) and carcinomas. Endoscopy is the usual method employed to diagnose these condition. Another noninvasive way to assess and diagnose GI conditions are fecal biomarkers. Fecal biomarkers provide information regarding a specific disease process and are perhaps more acceptable to clinicians and patients alike because of their non-invasivity compared to endoscopy. Aim of this review was to evaluate the current status of the fecal biomarkers in clinical and research for in GI diseases...
February 6, 2017: World Journal of Gastrointestinal Pharmacology and Therapeutics
https://www.readbyqxmd.com/read/28203073/health-status-and-lung-function-in-the-swedish-alpha-1-antitrypsin-deficient-cohort-identified-by-neonatal-screening-at-the-age-of-37-40-years
#3
Eeva Piitulainen, Behrouz Mostafavi, Hanan A Tanash
BACKGROUND: Severe alpha 1-antitrypsin (AAT) deficiency (genotype PiZZ) is a well-known risk factor for COPD. A cohort of PiZZ and PiSZ individuals was identified by the Swedish national neonatal AAT screening program in 1972-1974 and followed up regularly since birth. Our aim was to study the lung function, respiratory symptoms and health status at the age of 38 years in comparison with a random sample of control subjects selected from the population registry. METHODS: The study group included 120 PiZZ, 46 PiSZ and 164 control subjects (PiMM), who answered a questionnaire on smoking habits and symptoms and the Saint George Respiratory Questionnaire (SGRQ) on quality of life...
2017: International Journal of Chronic Obstructive Pulmonary Disease
https://www.readbyqxmd.com/read/28198571/pulmonary-mri-morphometry-modeling-of-airspace-enlargement-in-chronic-obstructive-pulmonary-disease-and-alpha-1-antitrypsin-deficiency
#4
Alexei Ouriadov, Eric Lessard, Khadija Sheikh, Grace Parraga
PURPOSE: We generated lung morphometry measurements using single-breath diffusion-weighted MRI and three different acinar duct models in healthy participants and patients with emphysema stemming from chronic obstructive lung disease (COPD) and alpha-1 antitrypsin deficiency (AATD). METHODS: Single-breath-inhaled (3) He MRI with five diffusion sensitizations (b-value = 0, 1.6, 3.2, 4.8, and 6.4 s/cm(2) ) was used, and signal intensities were fit using a cylindrical and single-compartment acinar-duct model to estimate MRI-derived mean linear intercept (Lm ) and surface-to-volume ratio (S/V)...
February 15, 2017: Magnetic Resonance in Medicine: Official Journal of the Society of Magnetic Resonance in Medicine
https://www.readbyqxmd.com/read/28191362/the-anti-inflammatory-effect-of-alpha-1-antitrypsin-in-rhinovirus-infected-human-airway-epithelial-cells
#5
Di Jiang, Reena Berman, Qun Wu, Connor Stevenson, Hong Wei Chu
OBJECTIVE: Excessive airway inflammation is seen in chronic obstructive pulmonary disease (COPD) patients experiencing acute exacerbations, which are often associated with human rhinovirus (HRV) infection. Alpha-1 antitrypsin (A1AT) has anti-inflammatory function in endothelial cells and monocytes, but its anti-inflammatory effect has not been investigated in COPD airway epithelial cells. We determined A1AT's anti-inflammatory function in COPD airway epithelial cells and the underlying mechanisms such as the role of caspase-1...
December 2016: Journal of Clinical & Cellular Immunology
https://www.readbyqxmd.com/read/28185876/associations-between-food-consumption-patterns-and-saliva-composition-specificities-of-eating-difficulties-children
#6
Martine Morzel, Caroline Truntzer, Eric Neyraud, Hélène Brignot, Patrick Ducoroy, Géraldine Lucchi, Cécile Canlet, Ségolène Gaillard, Florian Nicod, Sophie Nicklaus, Noël Peretti, Gilles Feron
Identifying objective markers of diet would be beneficial to research fields such as nutritional epidemiology. As a preliminary study on the validity of using saliva for this purpose, and in order to explore the relationship between saliva and diet, we focused on clearly contrasted groups of children: children with eating difficulties (ED) receiving at least 50% of their energy intake through artificial nutrition vs healthy controls (C). Saliva of ED and C children was analyzed by various methods (targeted biochemical analyses, 2-D electrophoresis coupled to MS, (1)H NMR) and their diet was characterized using food frequency questionnaires, considering 148 food items grouped into 13 categories...
February 6, 2017: Physiology & Behavior
https://www.readbyqxmd.com/read/28183045/new-process-for-purifying-high-purity-%C3%AE-1-antitrypsin-from-cohn-fraction-iv-by-chromatography-a-promising-method-for-the-better-utilization-of-plasma
#7
Chaoji Huangfu, Jinchao Zhang, Yuyuan Ma, Junting Jia, Maomin Lv, Xiong Zhao, Jingang Zhang
α1-antitrypsin (AAT) is a 52kDa serine protease inhibitor that is abundant in plasma. It is synthesized mainly by hepatic cells, and widely used to treat patients with emphysema due to congenital deficiency of AAT. A new isolation method for the purification of AAT from Cohn Fraction IV (Cohn F IV) is described. Cohn F IV is usually discarded as a byproduct from Cohn process. Using Cohn F IV as starting material does not interfere with the production of other plasma proteins and the cost of purification could be reduced greatly...
February 1, 2017: Journal of Chromatography. B, Analytical Technologies in the Biomedical and Life Sciences
https://www.readbyqxmd.com/read/28182151/patterns-and-characterization-of-copd-exacerbations-using-real-time-data-collection
#8
Stanley I Ejiofor, Jan Stolk, Pablo Fernandez, Robert A Stockley
INTRODUCTION: Patients with chronic obstructive pulmonary disease often experience exacerbations. These events are important as they are a major cause of morbidity and mortality. Recently, it has been increasingly recognized that patients may experience symptoms suggestive of an exacerbation but do not seek treatment, which are referred to as unreported or untreated exacerbations. Symptom diaries used in clinical trials have the benefit of identifying both treated and untreated exacerbation events...
2017: International Journal of Chronic Obstructive Pulmonary Disease
https://www.readbyqxmd.com/read/28178162/genotyping-diagnosis-of-alpha-1-antitrypsin-deficiency-in-saudi-adults-with-liver-cirrhosis
#9
Noura Al-Jameil, Amina A Hassan, Ahlam Buhairan, Rana Hassanato, Sree R Isac, Maram Al-Otaiby, Basmah Al-Maarik, Iman Al-Ajeyan
The acute phase protein alpha-1 antitrypsin (AAT) is mainly produced in liver cells. AAT deficiency affects the lungs and liver. We conducted a case-control study to define a valuable method for the proper diagnosis of alpha-1 antitrypsin deficiency (AATD), as well as the association of liver cirrhosis with AATD in Saudi adults.Blood samples from 300 liver cirrhosis patients and 400 controls were analyzed according to serum AAT concentration, phenotyping, and genotyping. Nephelometry was used for AAT quantification, isoelectric focusing electrophoresis was used for phenotyping detection, and real-time PCR was used for genotyping to determine the Z and S deficiency alleles...
February 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28177773/gastroprotective-and-antielastase-effect-of-protein-inhibitors-from-erythrina-velutina-seeds-in-an-experimental-ulcer-model
#10
Vanessa Cristina Oliveira de Lima, Richele Janaína de Araújo Machado, Norberto Monteiro, Christina da Silva Camillo, Alexandre Coelho Serquiz, Adeliana Silva de Oliveira, Fabiola Rufino, Bruna Leal Lima Maciel, Adriana Ferreira Uchôa, Elizeu Antunes Santos, Ana Heloneida de Araújo Morais
Trypsin and chymotrypsin inhibitors from Erythrina velutina seeds have been previously isolated by our group. In previous studies using a sepsis model, we demonstrated the anti-tumor and anti-inflammatory action of these compounds. This study aimed to evaluate the gastroprotective and antielastase effect of protein inhibitors from Erythrina velutina seeds in an experimental stress induced ulcer model. Two protein isolates from E. velutina seeds, with antitrypsin (PIAT) and anti-chymotrypsin (PIAQ) activities were tested...
September 12, 2016: Biochemistry and Cell Biology, Biochimie et Biologie Cellulaire
https://www.readbyqxmd.com/read/28158993/comparative-differential-proteomic-analysis-of-minimal-change-disease-and-focal-segmental-glomerulosclerosis
#11
Vanessa Pérez, Dolores López, Ester Boixadera, Meritxell Ibernón, Anna Espinal, Josep Bonet, Ramón Romero
BACKGROUND: Minimal change disease (MCD) and primary focal segmental glomerulosclerosis (FSGS) are glomerular diseases characterized by nephrotic syndrome. Their diagnosis requires a renal biopsy, but it is an invasive procedure with potential complications. In a small biopsy sample, where only normal glomeruli are observed, FSGS cannot be differentiated from MCD. The correct diagnosis is crucial to an effective treatment, as MCD is normally responsive to steroid therapy, whereas FSGS is usually resistant...
February 3, 2017: BMC Nephrology
https://www.readbyqxmd.com/read/28144858/a-case-of-adult-undifferentiated-embryonal-sarcoma-of-the-liver-successfully-treated-with-right-trisectionectomy-a-case-report
#12
Akiko Mori, Koji Fukase, Kunihiro Masuda, Naoaki Sakata, Masamichi Mizuma, Hideo Ohtsuka, Takanori Morikawa, Kei Nakagawa, Hiroki Hayashi, Fuyuhiko Motoi, Takeshi Naitoh, Keigo Murakami, Michiaki Unno
BACKGROUND: Undifferentiated embryonal sarcoma of the liver (UESL) is a rare malignant mesenchymal tumor that usually occurs in children and is rarely diagnosed in adults. CASE PRESENTATION: Here, we describe the case of a 65-year-old woman who presented with a huge cystic lesion in the liver. Laboratory studies performed on admission showed modest inflammation, poor nutrition, and elevated levels of total bilirubin, alkaline phosphatase, and γ-glutamyl transferase...
December 2017: Surgical Case Reports
https://www.readbyqxmd.com/read/28142234/plasma-protein-binding-structure-activity-relationships-related-to-the-n-terminus-of-daptomycin
#13
Elena K Schneider, Johnny X Huang, Vincenzo Carbone, Meiling Han, Yan Zhu, Sue Nang, Keith K Khoo, Johnson Mak, Matthew A Cooper, Jian Li, Tony Velkov
Daptomycin is a lipopeptide antibiotic that is highly bound to plasma proteins. To date, the plasma components and structure-activity relationships responsible for the plasma protein binding profile of daptomycin remain uncharacterized. In the present study we have employed a surface plasmon resonance assay together with molecular docking techniques to investigate the plasma protein binding structure-activity relationships related to the N-terminal fatty acyl of daptomycin. Three compounds were investigated: (1) native daptomycin, which displays an N-terminal n-decanoyl fatty acid side chain, and two analogues with modifications to the N-terminal fatty acyl chain; (2) des-acyl daptomycin; and (3) acetyl-daptomycin...
February 10, 2017: ACS Infectious Diseases
https://www.readbyqxmd.com/read/28137880/class-i-restricted-t-cell-responses-to-a-polymorphic-peptide-in-a-gene-therapy-clinical-trial-for-%C3%AE-1-antitrypsin-deficiency
#14
Roberto Calcedo, Suryanarayan Somanathan, Qiuyue Qin, Michael R Betts, Andrew J Rech, Robert H Vonderheide, Christian Mueller, Terence R Flotte, James M Wilson
Adeno-associated virus (AAV)-mediated gene therapy is currently being pursued as a treatment for the monogenic disorder α-1-antitrypsin (AAT) deficiency. Results from phase I and II studies have shown relatively stable and dose-dependent increases in transgene-derived wild-type AAT after local intramuscular vector administration. In this report we describe the appearance of transgene-specific T-cell responses in two subjects that were part of the phase II trial. The patient with the more robust T-cell response, which was associated with a reduction in transgene expression, was characterized more thoroughly in this study...
January 30, 2017: Proceedings of the National Academy of Sciences of the United States of America
https://www.readbyqxmd.com/read/28129926/panniculitis-a-summary
#15
Mark R Wick
The diagnosis of panniculitis is felt to be a confusing topic by some pathologists. This summarical article presents inflammatory diseases of the subcutis in a systematic fashion, based on whether they are centered on fibrovascular septa or the adipose lobules, and whether morphologic vasculitis is present or not. Septocentric, non-vasculitis disorders include erythema nodosum, panniculitis that follows the use of "biological" therapeutic agents, lipodermatosclerosis, post-irradiation panniculitis, morphea profunda, and necrobiosis lipodica profunda...
December 27, 2016: Seminars in Diagnostic Pathology
https://www.readbyqxmd.com/read/28121484/ubiquitin-ligase-syvn1-hrd1-facilitates-degradation-of-the-serpina1-z-variant-alpha-1-antitrypsin-z-variant-via-sqstm1-p62-dependent-selective-autophagy
#16
Lijie Feng, Jin Zhang, Na Zhu, Qian Ding, Xiaojie Zhang, Jishuang Yu, Weimin Qiang, Zhetao Zhang, Yuyang Ma, Dake Huang, Yujun Shen, Shengyun Fang, Yifan Yu, Haiping Wang, Yuxian Shen
SERPINA1/AAT/alpha-1-antitrypsin (serpin family A member 1) deficiency (SERPINA1/ AAT-D) is an autosomal recessive disorder characterized by the retention of misfolded SERPINA1/AAT in the endoplasmic reticulum (ER) of hepatocytes and a significant reduction of serum SERPINA1/AAT level. The Z variant of SERPINA1/AAT, containing a Glu342Lys (E342K) mutation (SERPINA1(E342K)/ATZ), the most common form of SERPINA1/AAT-D, is prone to misfolding and polymerization, which retains it in the ER of hepatocytes and leads to liver injury...
January 25, 2017: Autophagy
https://www.readbyqxmd.com/read/28120746/state-of-the-art-testing-for-alpha-1-antitrypsin-deficiency
#17
F Kueppers, C Sanders
BACKGROUND: Alpha-1 antitrypsin deficiency (AATD) is a genetic condition characterized by low serum levels of the proteinalpha-1 antitrypsin. Because there are no unique clinical symptoms that point to a definitive diagnosis of AATD, laboratorytesting is crucial to differentiate this disease from others. OBJECTIVE: To summarize advances in laboratory techniques used to test for AATD. METHODS: Data were sourced from a nonsystematic literature review of MEDLINE and the author's personal literature collection, and by checking reference lists of sourced articles...
January 24, 2017: Allergy and Asthma Proceedings:
https://www.readbyqxmd.com/read/28117336/cd5-molecule-like-and-transthyretin-as-putative-biomarkers-of-chronic-myeloid-leukemia-an-insight-from-the-proteomic-analysis-of-human-plasma
#18
Iram Fatima, Saima Sadaf, Syed Ghulam Musharraf, Naghma Hashmi, Muhammad Waheed Akhtar
Better and sensitive biomarkers are needed to help understand the mechanism of disease onset, progression, prognosis and monitoring of the therapeutic response. Aim of this study was to identify the candidate circulating markers of chronic-phase chronic myeloid leukemia (CP-CML) manifestations, having potential to develop into predictive- or monitoring-biomarkers. A proteomic approach, two-dimensional gel electrophoresis in conjunction with mass spectrometry (2DE-MS), was employed for this purpose. Based on the spot intensity measurements, six proteins were found to be consistently dysregulated in CP-CML subjects compared to the healthy controls [false discovery rate (FDR) threshold ≤0...
January 24, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28108341/molecular-cloning-genomic-structure-polymorphism-analysis-and-recombinant-expression-of-a-%C3%AE-1-antitrypsin-like-gene-from-swamp-eel-monopterus-albus
#19
Wei Li, Quanhe Wang, Shaobin Li, Ao Jiang, Wenxiu Sun
Alpha-1-antitrypsin (AAT) is a highly polymorphic glycoprotein antiprotease, involved in the regulation of human immune response. Beyond some genomic characterization and a few protein characterizations, the function of teleost AAT remains uncertain. In this study we cloned an AAT-like gene from a swamp eel liver identifying four exons and three introns, and the full-length cDNA. The elucidated swamp eel AAT amino acid sequence showed high homology with known AATs from other teleosts. The swamp eel AAT was examined both in ten healthy tissues and in four bacterially-stimulated tissues resulting in up-regulation of swamp eel AAT at different times...
January 17, 2017: Fish & Shellfish Immunology
https://www.readbyqxmd.com/read/28107454/identification-of-novel-short-c-terminal-transcripts-of-human-serpina1-gene
#20
Nerea Matamala, Nupur Aggarwal, Paolo Iadarola, Marco Fumagalli, Gema Gomez-Mariano, Beatriz Lara, Maria Teresa Martinez, Isabel Cuesta, Jan Stolk, Sabina Janciauskiene, Beatriz Martinez-Delgado
Human SERPINA1 gene is located on chromosome 14q31-32.3 and is organized into three (IA, IB, and IC) non-coding and four (II, III, IV, V) coding exons. This gene produces α1-antitrypsin (A1AT), a prototypical member of the serpin superfamily of proteins. We demonstrate that human peripheral blood leukocytes express not only a product corresponding to the transcript coding for the full-length A1AT protein but also two short transcripts (ST1C4 and ST1C5) of A1AT. In silico sequence analysis revealed that the last exon of the short transcripts contains an Open Reading Frame (ORF) and thus putatively can produce peptides...
2017: PloS One
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