Read by QxMD icon Read

Alpha 1 antitrypsin

Kay Weng Choy, James C G Doery, Nilika Wijeratne
No abstract text is available yet for this article.
February 2016: Pathology
Maria Paula Henao, Timothy J Craig
Alpha-1-antitrypsin Deficiency (AATD) is an orphan disease that predisposes individuals to COPD and liver disease. The following is a comprehensive review of AATD from epidemiology to treatment for physicians who treat COPD or asthma. Areas Covered: In this comprehensive review of alpha-1-antitrypsin deficiency, we describe the historical perspective, genetics, epidemiology, clinical presentation and symptoms, screening and diagnosis, and treatments of the condition. Expert Commentary: The two most important directions for advancing the understanding of AATD involve improving detection of the condition, especially in asymptomatic patients, and advancing knowledge of treatments directed specifically at AATD-related conditions...
October 24, 2016: Expert Review of Respiratory Medicine
L Renee Ruhaak
In recent years, high-throughput glycomics approaches have been developed and applied to either complete biofluids, cell lysates or tissues, or proteins isolated thereof. However, during such analyses the N-glycan are released from the protein backbone and therefore site- and protein-specific information is lost. There exists a need for high-throughput methods that allow quantification of site- and protein-specific glycosylation patterns from complex biological mixtures. We here describe the use of a multiple reaction monitoring mass spectrometry based method for the generation of glycopeptide profiles of the nine high abundance glycoproteins IgG, IgA, IgM, haptoglobin, alpha-1-antitrypsin, alpha-2-macroglobulin, alpha-1-acid glycoprotein, transferrin, and complement C3...
2017: Methods in Molecular Biology
I Tuleta, F Stöckigt, U R Juergens, C Pizarro, J W Schrickel, G Kristiansen, G Nickenig, D Skowasch
INTRODUCTION: Intermittent hypoxia as a surrogate of obstructive sleep apnea is associated with different cardiovascular complications. However, the effects of intermittent hypoxia on the lung tissue are less known. Therefore, the aim of our present study was to investigate if intermittent hypoxia may influence oxidative stress, inflammation, and protease/antiprotease system in the lung. Additionally, potential protective properties of anti-inflammatory and anti-oxidative drugs have been evaluated...
October 13, 2016: Lung
Kumar Kanishka, Anand Yogeshman, Jha Kumar Rajendra, Kachhap Sushil
No abstract text is available yet for this article.
January 2016: Journal of the Association of Physicians of India
Anna Sroka-Bartnicka, Isabella Karlsson, Lorena Ndreu, Alessandro Quaranta, Matthijs Pijnappel, Gunnar Thorsén
Glycosylation is one of the most common and important post-translational modifications, influencing both the chemical and the biological properties of proteins. Studying the glycosylation of the entire protein population of a sample can be challenging because variations in the concentrations of certain proteins can enhance or obscure changes in glycosylation. Furthermore, alterations in the glycosylation pattern of individual proteins, exhibiting larger variability in disease states, have been suggested as biomarkers for different types of cancer, as well as inflammatory and neurodegenerative diseases...
October 3, 2016: Journal of Pharmaceutical and Biomedical Analysis
Limin Hao, Oshrit Ben-David, Suzann M Babb, Anthony H Futerman, Bruce M Cohen, Edgar A Buttner
Defining the mechanisms of action of the anti-psychotic drug (APD), clozapine is of great importance, as clozapine is more effective and has therapeutic benefits in a broader range of psychiatric disorders than other APDs. Its range of actions have not been fully characterized. Exposure to APDs early in development causes dose-dependent developmental delay and lethality in C. elegans. A previous genome-wide RNAi screen for suppressors of clozapine-induced developmental delay and lethality revealed 40 candidate genes, including sms-1, which encodes a sphingomyelin synthase...
October 6, 2016: Neuropsychopharmacology: Official Publication of the American College of Neuropsychopharmacology
Nikolas Bernhard, Robert Bals, Sebastian Fähndrich
Alpha-1-antitrypsin deficiency is a genetic risk factor for the development of chronic obstructive airway disease (COPD) and liver cirrhosis. The disease is widely underdiagnosed. The hallmarks of therapy are smoking cessation, prevention from environmental dust exposure and augmentation therapy. Findings from the recently published prospective, placebo-controlled and randomized RAPID trial proved effectiveness of AAT augmentation therapy for slowing progression of emphysema, measured by CT lung density. CT lung density may be more sensitive than forced exspiratory volume in one second (FEV1) or monoxid diffusion capacity (DLCO)...
September 2016: Deutsche Medizinische Wochenschrift
Luis Sendra, Antonio Miguel, Daniel Pérez-Enguix, María José Herrero, Eva Montalvá, María Adelaida García-Gimeno, Inmaculada Noguera, Ana Díaz, Judith Pérez, Pascual Sanz, Rafael López-Andújar, Luis Martí-Bonmatí, Salvador F Aliño
INTRODUCTION: Expressing exogenous genes after naked DNA delivery into hepatocytes might achieve sustained and high expression of human proteins. Tail vein DNA injection is an efficient procedure for gene transfer in murine liver. Hydrodynamic procedures in large animals require organ targeting, and improve with liver vascular exclusion. In the present study, two closed liver hydrofection models employing the human alpha-1-antitrypsin (hAAT) gene are compared to reference standards in order to evaluate their potential clinical interest...
2016: PloS One
Marcin Surmiak, Marek Sanak
BACKGROUND: One of characteristic findings in granulomatosis with polyangiitis (GPA) is the presence of proteinase-3 (anti-PR3) specific antibodies. These antibodies can cause neutrophil activation, degranulation and generation of reactive oxygen species (ROS). Each of these inflammatory events can be suppressed by circulating alpha-1 antitrypsin (A1AT). A1AT is an acute phase protein increasing during inflammation, however, it may circulate as an inactive polymeric protein. AIM OF THE STUDY: The aim was to analyze how different types of A1AT can affect anti-PR3 mediated neutrophil activation...
August 5, 2016: Pharmacological Reports: PR
Shih-Yi Lin, Chao-Hsiang Chang, His-Chin Wu, Ching-Chan Lin, Kai-Po Chang, Chi-Rei Yang, Chi-Ping Huang, Wu-Huei Hsu, Chiz-Tzung Chang, Chao-Jung Chen
MALDI-TOF spectrometry has not been used for urinary exosome analysis. We used it for determining UC biomarkers. From 2012 to 2015, we enrolled 129 consecutive patients with UC and 62 participants without UC. Exosomes from their urine were isolated, and analyzed through MALDI-TOF spectrometry. Immunohistochemical (IHC) analysis of another 122 UC and 26 non-UC tissues was conducted to verify the discovered biomarkers. Two peaks at m/z 5593 (fragmented peptide of alpha-1-antitrypsin; sensitivity, 50.4%; specificity, 96...
September 30, 2016: Scientific Reports
Inga Jarosch, Sebastian Gehlert, Daniel Jacko, Rembert Andreas Koczulla, Marion Wencker, Tobias Welte, Wilhelm Bloch, Sabina Janciauskiene, Klaus Kenn
BACKGROUND: Pulmonary rehabilitation (PR) improves oxidative capacity of peripheral muscles in patients with chronic obstructive pulmonary disease (COPD). The exercise-induced oxidative skeletal muscle adaptation in COPD patients with inherited alpha-1 antitrypsin deficiency (A1ATD) has not been studied. OBJECTIVES: To compare PR effects on skeletal muscle adaptation in COPD patients with and without A1ATD. METHODS: Nine COPD patients with A1ATD (genotype PiZZ, 6 receiving A1AT augmentation therapy), and 10 'usual' COPD patients (genotype PiMM) performed an incremental cycling test and underwent musculus vastus lateralis biopsies before and after a 3-week PR program including exercise training...
September 30, 2016: Respiration; International Review of Thoracic Diseases
Mardiaty Iryani Abdullah, Ching Chin Lee, Sarni Mat Junit, Khoon Leong Ng, Onn Haji Hashim
BACKGROUND: Papillary thyroid cancer (PTC) is mainly diagnosed using fine-needle aspiration biopsy. This most common form of well-differentiated thyroid cancer occurs with or without a background of benign thyroid goiter (BTG). METHODS: In the present study, a gel-based proteomics analysis was performed to analyse the expression of proteins in tissue and serum samples of PTC patients with (PTCb; n = 6) and without a history of BTG (PTCa; n = 8) relative to patients with BTG (n = 20)...
2016: PeerJ
Adel Boueiz, Sharon M Lutz, Michael H Cho, Craig P Hersh, Russell P Bowler, George R Washko, Eitan Halper-Stromberg, Per Bakke, Amund Gulsvik, Nan M Laird, Terri H Beaty, Harvey O Coxson, James D Crapo, Edwin K Silverman, Peter J Castaldi, Dawn L DeMeo
RATIONALE: Emphysema has considerable variability in the severity and distribution of parenchymal destruction throughout the lungs. Upper lobe predominant emphysema has emerged as an important predictor of response to lung volume reduction surgery. Yet, aside from alpha-1 antitrypsin deficiency, the genetic determinants of emphysema distribution remain largely unknown. OBJECTIVES: To identify the genetic influences of emphysema distribution in non-alpha-1 antitrypsin deficient smokers...
September 26, 2016: American Journal of Respiratory and Critical Care Medicine
Peter C Gøtzsche, Helle Krogh Johansen
BACKGROUND: Alpha-1 antitrypsin deficiency is an inherited disorder that can cause chronic obstructive pulmonary disease (COPD). People who smoke are more seriously affected and have a greater risk of dying from the disease. Therefore, the primary treatment is to help people give up smoking. There are now also preparations available that contain alpha-1 antitrypsin, but it is uncertain what their clinical effect is. OBJECTIVES: To review the benefits and harms of augmentation therapy with intravenous alpha-1 antitrypsin in patients with alpha-1 antitrypsin deficiency and lung disease...
September 20, 2016: Cochrane Database of Systematic Reviews
Napat Angkathunyakul, Francesca Rosini, Nigel Heaton, Pierre Foskett, Alberto Quaglia
AIMS: Both homo- and heterozygous AAT deficiency patients are risk of hepatocellular carcinoma (HCC) but also of cholangiocarcinoma and combined hepatocellular-cholangiocarcinoma. We report a series of bile duct adenoma (BDA) and intrahepatic cholangiocarcinoma (ICC), in AAT deficiency adult patients, observed in our institution over a five year period. Our observational study includes a detailed investigation of their immunohistochemical profile and BRAF V600E mutation status. METHODS AND RESULTS: Eleven biliary lesions from 5 AAT deficiency patients (6 BDAs from 3 cirrhotic patients with concurrent other liver diseases, 3 BDAs and 2 ICCs from 2 non-cirrhotic patients) were identified between 2010 and 2015 during routine histology...
September 14, 2016: Histopathology
Mila Ljujic, Sanja Mijatovic, Mirna Z Bulatovic, Marija Mojic, Danijela Maksimovic-Ivanic, Dragica Radojkovic, Aleksandra Topic
Increased circulating alpha-1-antitrypsin (AAT) correlates with cancer stage/aggressiveness, but its role in cancer biology is unclear. We revealed antagonistic effect of AAT to cisplatin-induced cytotoxicity in prostate (PC3) and melanoma (A375) cancer cell lines. Moreover, AAT abrogated cytotoxicity of MEK inhibitor U0126 in PC3 cell line. Weaker antagonistic effect of AAT on cytotoxicity of PI3/Akt and NF-kB inhibitors was also observed. In addition, cisplatin increased AAT gene expression in transfected PC3 cells...
September 12, 2016: Pathology Oncology Research: POR
J Stollenwerk, M Schepke, E Biecker
BACKGROUND: Altered liver function tests are a common finding in clinical practice. Our retrospective study aimed to identify the diagnoses in a non-selected cohort of patients with altered liver tests and to investigate whether alpha-1 antitrypsin genotyping should be part of the diagnostic workup. PATIENTS AND METHODS: 501 patients who were admitted to our outpatient clinic for further evaluation of altered liver function tests were included in the study. The patients underwent a standardized diagnostic program with history taking, physical examination, laboratory tests and ultrasonography...
September 2016: Zeitschrift Für Gastroenterologie
Romana Raphaela Gerner, Clemens Feistritzer, Alexander Rupert Moschen, Brigitte Kircher, Patrizia Moser, Herbert Tilg, David Nachbaur
No abstract text is available yet for this article.
September 8, 2016: Transplantation
Jennifer A Dickens, Adriana Ordóñez, Joseph E Chambers, Alison J Beckett, Vruti Patel, Elke Malzer, Caia S Dominicus, Jayson Bradley, Andrew A Peden, Ian A Prior, David A Lomas, Stefan J Marciniak
Alpha-1-antitrypsin is a serine protease inhibitor produced in the liver that is responsible for the regulation of pulmonary inflammation. The commonest pathogenic gene mutation yields Z-α1-antitrypsin, which has a propensity to self-associate forming polymers that become trapped in inclusions of endoplasmic reticulum (ER). It is unclear whether these inclusions are connected to the main ER network in Z-α1-antitrypsin-expressing cells. Using live cell imaging, we found that despite inclusions containing an immobile matrix of polymeric α1-antitrypsin, small ER resident proteins can diffuse freely within them...
September 6, 2016: FASEB Journal: Official Publication of the Federation of American Societies for Experimental Biology
Fetch more papers »
Fetching more papers... Fetching...
Read by QxMD. Sign in or create an account to discover new knowledge that matter to you.
Remove bar
Read by QxMD icon Read

Search Tips

Use Boolean operators: AND/OR

diabetic AND foot
diabetes OR diabetic

Exclude a word using the 'minus' sign

Virchow -triad

Use Parentheses

water AND (cup OR glass)

Add an asterisk (*) at end of a word to include word stems

Neuro* will search for Neurology, Neuroscientist, Neurological, and so on

Use quotes to search for an exact phrase

"primary prevention of cancer"
(heart or cardiac or cardio*) AND arrest -"American Heart Association"