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Alpha 1 antitrypsin

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https://www.readbyqxmd.com/read/29351445/impact-of-hiv-infection-on-alpha-1-antitrypsin-in-the-lung
#1
Sarah E Stephenson, Carole L Wilson, Kristina Crothers, Engi F Attia, Cherry Wongtrakool, Irina Petrache, Lynn M Schnapp
BACKGROUND: Emphysema is one of the most common lung diseases in HIV+ individuals. The pathogenesis of HIV-associated emphysema remains unclear; however, radiographic distribution and earlier age of presentation of emphysema in the lungs of HIV+ patients are similar to deficiency of alpha-1 antitrypsin (A1AT), a key elastase inhibitor in the lung. Reduced levels of circulating A1AT have been detected in HIV+ patients, suggesting a potential mechanism for emphysema development. In the present study, we asked if A1AT levels and activity in the bronchoalveolar lavage fluid (BALF) differ in patients with and without HIV and emphysema...
December 14, 2017: American Journal of Physiology. Lung Cellular and Molecular Physiology
https://www.readbyqxmd.com/read/29348884/alpha-1-antitrypsin-functions-as-a-protective-factor-in-preeclampsia-through-activating-smad2-and-inhibitor-of-dna-binding-4
#2
Yaling Feng, Nan Wang, Jianjuan Xu, Jinfang Zou, Xi Liang, Huan Liu, Ying Chen
Pre-eclampsia (PE) is one of the most common reason for high morbidity and mortality of maternal and prenatal infants. Production from oxidative stress results in maternal ROS system and anti-oxidation defense system imbalance to promote tissue ischemia and hypoxia, and ultimately impairs the maternal organs and placenta. Our previous study showed that exogenous Alpha-1-antitrypsin (AAT) and overexpression of AAT in umbilical vein cell (HUVEC) hypoxia-reoxygenation model could increase the activity of antioxidant enzymes, and played a protective role in preeclampsia animal model...
December 22, 2017: Oncotarget
https://www.readbyqxmd.com/read/29289686/the-therapeutic-potential-of-hyaluronan-in-copd
#3
Gerard M Turino, Shuren Ma, Yong Y Lin, Jerome O Cantor
Insights into the clinical course in COPD indicate the need for new therapies in COPD. The discovery of alpha-1 antitrypsin deficiency (AATD) led to the protease-antiprotease imbalance hypothesis which was applied to COPD related and not related to AATD. The discovery of AATD brought recognition to the importance of elastin fibers in maintaining lung matrix structure. Two cross-linking amino acids are unique to mature elastin, desmosine and isodesmosine (DI), which can serve as biomarkers of the degradation of elastin...
December 28, 2017: Chest
https://www.readbyqxmd.com/read/29282492/the-use-of-fecal-calprotectin-levels-in-the-fontan-population
#4
Carlos Miranda, Anas Taqatqa, Adrian Chapa-Rodriguez, Jacob P Holton, Sawsan M Awad
The Fontan procedure was first performed in the seventies as a palliation for patients with single ventricle physiology. A feared complication after a Fontan procedure is the development of protein losing enteropathy (PLE). Systemic inflammation has a negative effect on the intestinal barrier integrity, which has supported the use of steroids in this setting. To the best of our knowledge there are no studies linking intestinal inflammation in patients with PLE after Fontan. The objective of this study was to identify the presence of intestinal inflammation measured by FC in patients with PLE after a Fontan procedure...
December 28, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/29259521/nuclease-mediated-gene-therapies-for-inherited-metabolic-diseases-of-the-liver%C3%A2-%C3%A2
#5
REVIEW
Taylor E Bryson, Caitlin M Anglin, P Hudson Bridges, Renee N Cottle
Inherited metabolic diseases (IMDs) of the liver represent a vast and diverse group of rare genetic diseases characterized by the loss or dysfunction of enzymes or proteins essential for metabolic pathways in the liver. Conventional gene therapy involving adeno-associated virus (AAV) serotype 8 vectors provide therapeutically high levels of hepatic transgene expression facilitating the correction of the disease phenotype in pre-clinical studies and are currently being evaluated in clinical trials for multiple IMDs...
December 2017: Yale Journal of Biology and Medicine
https://www.readbyqxmd.com/read/29233247/pelvic-organ-prolapse-in-a-fighter-pilot-with-alpha-1-antitrypsin-deficiency
#6
Kerry Buckley, Jami Gann, Heather Barbier, Joy Greer
BACKGROUND: Few researchers have studied symptomatic younger to middle-aged women with pelvic organ prolapse. The association between highly strenuous activity and prolapse symptoms is largely theoretical. It is also known that a genetic component likely contributes to early-onset pelvic organ prolapse, but prevention and treatment with respect to this has not been explored. Service restrictions differ for active duty women who are diagnosed with symptomatic pelvic organ prolapse. CASE REPORT: We present a case of a 33-yr-old Gravida 1 Para 1 fighter pilot who developed symptomatic pelvic organ prolapse after a vaginal delivery...
January 1, 2018: Aerospace Medicine and Human Performance
https://www.readbyqxmd.com/read/29232161/characterization-of-novel-missense-variants-of-serpina1-gene-causing-alpha-1-antitrypsin-deficiency
#7
Nerea Matamala, Beatriz Lara, Gema Gomez-Mariano, Selene Martínez, Diana Retana, Taiomara Fernandez, Ramona Angeles Silvestre, Irene Belmonte, Francisco Rodriguez-Frias, Marçal Vilar, Raquel Sáez, Igor Iturbe, Silvia Castillo, María Molina-Molina, Anna Texido, Gema Tirado-Conde, Jose Luis Lopez-Campos, Manuel Posada, Ignacio Blanco, Sabina Janciauskiene, Beatriz Martinez-Delgado
SERPINA1 gene is highly polymorphic, with more than one hundred variants described in databases. The SERPINA1 encodes alpha-1 antitrypsin (AAT) protein, and the severe deficiency of AAT is a major contributor to pulmonary emphysema and liver diseases. We report seven new variants in Spanish patients with AAT deficiency. All variants involved amino acid substitutions in different exons: PiSDonosti (S+Ser14Phe), PiTijarafe (Ile50Asn), PiSevilla (Ala58Asp), PiCadiz (Glu151Lys), PiTarragona (Phe227Cys), PiPuerto Real (Thr249Ala) and PiValencia (Lys328Glu)...
December 12, 2017: American Journal of Respiratory Cell and Molecular Biology
https://www.readbyqxmd.com/read/29227673/crystal-structure-of-cleaved-serp-1-a-myxomavirus-derived-immune-modulating-serpin-structural-design-of-serpin-reactive-center-loop-rcl-peptides-with-improved-therapeutic-function
#8
Brian P Mahon, Sriram Ambadapadi, Jordan R Yaron, Carrie L Lomelino, Melissa A Pinard, Shahar Keinan, Igor Kurnikov, Colin Macaulay, Liqiang Zhang, Westley Reeves, Grant McFadden, Scott Tibbetts, Robert McKenna, Alexandra Lucas
The Myxomavirus-derived protein Serp-1 has potent anti-inflammatory activity in models of vasculitis, lupus, viral sepsis, and transplant. Serp-1 has also been tested successfully in a Phase IIa clinical trial in unstable angina, representing a 'first-in-class' therapeutic. Recently, peptides derived from the reactive center loop (RCL) have been developed as stand-alone therapeutics for reducing vasculitis and improving survival in MHV68-infected mice. However, both Serp-1 and the RCL peptides lose activity in MHV68-infected mice after antibiotic suppression of intestinal microbiota...
December 11, 2017: Biochemistry
https://www.readbyqxmd.com/read/29225784/diagnosing-alpha-1-antitrypsin-deficiency-the-first-step-in-precision-medicine
#9
REVIEW
Craig P Hersh
Severe alpha-1 antitrypsin (AAT) deficiency is one of the most common serious genetic diseases in adults of European descent. Individuals with AAT deficiency have a greatly increased risk for emphysema and liver disease. Other manifestations include bronchiectasis, necrotizing panniculitis and granulomatosis with polyangiitis. Despite the frequency and potential severity, AAT deficiency remains under-recognized, and there is often a delay in diagnosis. This review will focus on three recent updates that should serve to encourage testing and diagnosis of AAT deficiency: first, the publication of a randomized clinical trial demonstrating the efficacy of intravenous augmentation therapy in slowing the progression of emphysema in AAT deficiency; second, the mounting evidence showing an increased risk of lung disease in heterozygous PI MZ genotype carriers; last, the recent publication of a clinical practice guideline, outlining diagnosis and management...
2017: F1000Research
https://www.readbyqxmd.com/read/29217818/proteomic-analysis-of-perfusate-from-machine-cold-perfusion-of-transplant-kidneys-insights-into-protection-from-injury
#10
Michael A J Moser, Katherine Sawicka, Steven Arcand, Preston O'Brien, Patrick Luke, Gavin Beck, Jolanta Sawicka, Alejandro Cohen, Grzegorz Sawicki
BACKGROUND Machine cold perfusion is beneficial to the preservation of kidneys for transplantation. At the end of preservation, the perfusion solution contains many proteins. Using a proteomics approach, we searched for useful biomarkers and potential therapeutic targets in the perfusate. Our program is unique in that all transplant kidneys (even living donor kidneys, LKD) are placed on machine cold perfusion prior to transplantation. MATERIAL AND METHODS Perfusates from donation after neurological and circulatory determination of death (DNDD and DCDD respectively) and LKD were collected (n=41) and analyzed for LDH, neutrophil gelatinase-associated lipocalin (NGAL), and matrix metalloproteinase-2 (MMP-2) as markers of injury...
December 8, 2017: Annals of Transplantation: Quarterly of the Polish Transplantation Society
https://www.readbyqxmd.com/read/29214586/epidemiology-of-rare-lung-diseases-the-challenges-and-opportunities-to-improve-research-and-knowledge
#11
Cormac McCarthy, Beatriz Lara Gallego, Bruce C Trapnell, Francis X McCormack
Rare lung diseases encompass a broad spectrum of conditions and affect an estimated 1.2-2.5 million people in North America and 1.5-3 million people in Europe. While individual rare lung diseases affect less than 1 in 2000 individuals, collectively they have a significant impact upon the population at large. Hence it is vital to understand firstly the epidemiology and subsequently the pathogenesis and clinical course of these disorders. Through a greater understanding of these aspects of disease, progress can be made in reducing symptoms, containing healthcare costs and utilizing resources efficiently...
2017: Advances in Experimental Medicine and Biology
https://www.readbyqxmd.com/read/29212317/targeted-measurements-of-o-and-n-glycopeptides-show-that-proteins-in-hdl-particles-are-enriched-with-specific-glycosylation-compared-to-plasma
#12
Muchena J Kailemia, Wanghui Wei, Khoa Nguyen, Elizabeth Beals, Lisa Sawrey-Kubicek, Christopher Rhodes, Chenghao Zhu, Romina Sacchi, Angela M Zivkovic, Carlito B Lebrilla
High density lipoprotein (HDL) particles are believed to be protective due to their inverse correlation with the prevalence of cardiovascular diseases. However, recent studies show that in some conditions such as heart disease and diabetes, HDL particles can become dysfunctional. Great attention has been directed toward HDL particle composition because the relative abundances of HDL constituents determine HDL's functional properties. A key factor to consider when studying the structure and composition of plasma particles is the protein glycosylation...
December 7, 2017: Journal of Proteome Research
https://www.readbyqxmd.com/read/29207246/multiple-reaction-monitoring-for-the-quantitation-of-serum-protein-glycosylation-profiles-application-to-ovarian-cancer
#13
Suzanne Miyamoto, Carol D Stroble, Sandra Taylor, Qiuting Hong, Carlito B Lebrilla, Gary S Leiserowitz, Kyoungmi Kim, L Renee Ruhaak
Protein glycosylation fingerprints are widely recognized as potential markers for disease states and indeed differential glycosylation has been identified in multiple types of autoimmune diseases and several types of cancer. However, releasing the glycans leave the glycoproteins unknown, therefore there exists a need for high-throughput methods that allow quantification of site- and protein- specific glycosylation patterns from complex biological mixtures. In this study, a targeted multiple reaction monitoring (MRM)-based method for the protein- and site- specific quantitation involving serum proteins immunoglobulins A, G and M, alpha-1-antitrypsin, transferrin, alpha-2-macroglobulin, haptoglobin, alpha-1-acid glycoprotein and complement C3 was developed...
December 5, 2017: Journal of Proteome Research
https://www.readbyqxmd.com/read/29206641/comparison-of-exercise-training-responses-in-copd-patients-with-and-without-alpha-1-antitrypsin-deficiency
#14
Inga Jarosch, Wolfgang Hitzl, Andreas Rembert Koczulla, Marion Wencker, Tobias Welte, Rainer Gloeckl, Sabina Janciauskiene, Klaus Kenn
BACKGROUND: The benefits of pulmonary rehabilitation (PR) on fatigue-resistant skeletal muscle fibre type I have been found to be smaller in COPD patients with alpha-1 antitrypsin deficiency (AATD) than in those without AATD. Alpha-1 antitrypsin (AAT) augmentation therapy was suggested as a potential factor of influence. Whether this finding mirrors different improvements in 6-min walk distance (6MWD) between both groups remains unknown. METHODS: 140 patients with AATD-related COPD (phenotype PiZZ, FEV1: 31 ± 8%pred...
September 2017: Respiratory Medicine
https://www.readbyqxmd.com/read/29206626/intensive-smoking-diminishes-the-differences-in-quality-of-life-and-exacerbation-frequency-between-the-alpha-1-antitrypsin-deficiency-genotypes-pizz-and-pisz
#15
Nikolas Bernhard, Philipp M Lepper, Claus Vogelmeier, Martina Seibert, Stefan Wagenpfeil, Robert Bals, Sebastian Fähndrich
BACKGROUND: Alpha-1-antitrypsin deficiency (AATD) is a rare genetic disorder that is associated with low levels of circulating alpha-1-antitrypsin in serum. In comparison to the genotype PiZZ, PiSZ usually leads to lower risk of emphysema, better lung function and better survival. The aim of this study was to analyze the relationship between cigarette smoking (packyears) and the AATD genotypes (PiZZ and PiSZ) concerning quality of life (SGRQ), transfer factor of the lung for carbon monoxide (TLCO), forced expiratory volume in one second (FEV1) and exacerbation rate...
September 2017: Respiratory Medicine
https://www.readbyqxmd.com/read/29192290/direct-conversion-of-human-fibroblasts-into-hepatocyte-like-cells-by-atf5-prox1-foxa2-foxa3-and-hnf4a-transduction
#16
Daiki Nakamori, Hiroki Akamine, Kazuo Takayama, Fuminori Sakurai, Hiroyuki Mizuguchi
Recently, it has been reported that human hepatocyte-like cells can be generated from fibroblasts by direct reprogramming technology. However, the conversion efficiency of human induced hepatocyte-like cells (hiHeps) is not high enough. In addition, comparative analysis with the existing models of hepatocytes, such as human iPS cell-derived hepatocyte-like cells and primary human hepatocytes, has not been sufficiently carried out. In this study, we screened hepatic transcription factors for efficient direct hepatic reprogramming and compared hepatic functions between hiHeps and other existing hepatocyte models...
November 30, 2017: Scientific Reports
https://www.readbyqxmd.com/read/29182883/clinical-experience-with-serpina1-dna-sequencing-to-detect-alpha-1-antitrypsin-deficiency
#17
François Maltais, Nathalie Gaudreault, Christine Racine, Sébastien Thériault, Yohan Bossé
No abstract text is available yet for this article.
November 28, 2017: Annals of the American Thoracic Society
https://www.readbyqxmd.com/read/29175361/the-role-of-computed-tomography-for-the-evaluation-of-lung-disease-in-alpha-1-antitrypsin-deficiency
#18
REVIEW
Michael A Campos, Alejandro A Diaz
Alpha-1 antitrypsin deficiency (AATD) is characterized by low serum levels of or dysfunctional alpha-1 proteinase inhibitor (A1-PI). In the lung parenchyma, this results in a loss of protection against the activity of serine proteases, particularly neutrophil elastase. The resultant imbalance in protease and anti-protease activity leads to an increased risk for the development of early-onset emphysema and chronic obstructive pulmonary disease (COPD). As in traditional smoke-related COPD, the assessment of the severity and disease progression of lung disease in AATD is conventionally based on lung function; however, pulmonary function tests are unable to discriminate between emphysema and airway disease, the two hallmark pathologic features of COPD...
November 23, 2017: Chest
https://www.readbyqxmd.com/read/29163550/evaluation-of-alpha-1-antitrypsin-levels-and-serpina1-gene-polymorphisms-in-sickle-cell-disease
#19
Magda Oliveira Seixas Carvalho, André Luís Carvalho Santos Souza, Mauricio Batista Carvalho, Ana Paula Almeida Souza Pacheco, Larissa Carneiro Rocha, Valma Maria Lopes do Nascimento, Camylla Vilas Boas Figueiredo, Caroline Conceição Guarda, Rayra Pereira Santiago, Adekunle Adekile, Marilda de Souza Goncalves
Alpha-1 antitrypsin (AAT) is an inhibitor of neutrophil elastase and a member of the serine proteinase inhibitor (serpin) superfamily, and little is known about its activity in sickle cell disease (SCD). We hypothesize that AAT may undergo changes in SCD because of the high oxidative stress and inflammation associated with the disease. We have found high AAT levels in SCD patients compared to controls, while mutant genotypes of SERPINA1 gene had decreased AAT levels, in both groups. AAT showed negative correlation with red blood cells, hemoglobin (Hb), hematocrit, high-density lipoprotein cholesterol, urea, creatinine, and albumin and was positively correlated with mean corpuscular Hb concentration, white blood cells, neutrophils, Hb S, bilirubin, lactate dehydrogenase, ferritin, and C-reactive protein...
2017: Frontiers in Immunology
https://www.readbyqxmd.com/read/29158881/serum-proteomic-profiling-of-obsessive-compulsive-disorder-washing-subtype-a-preliminary-study
#20
Mona Zamanian-Azodi, Mostafa Rezaei-Tavirani, Naser Nejadi, Afsaneh Arefi Oskouie, Faird Zayeri, Mostafa Hamdieh, Akram Safaei, Majid Rezaei-Tavirani, Alireza Ahmadzadeh, Alireza Amouzandeh-Nobaveh, Farshad Okhovatian
Introduction: Obsessive-Compulsive Disorder (OCD) is a disabling mental condition that its proteomic profiling is not yet investigated. Proteomics is a valuable tool to discover biomarker approaches. It can be helpful to detect protein expression changes in complex disorders such as OCD. Methods: Here, by the application of 2D gel electrophoresis (2DE), a pilot study of serum proteome profile of females with washing subtype of OCD was performed. Serum samples were obtained from females with washing subtype of OCD...
July 2017: Basic and Clinical Neuroscience
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