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https://www.readbyqxmd.com/read/29045001/anti-inflammaging-effects-of-human-alpha-1-antitrypsin
#1
Ye Yuan, Benedetto DiCiaccio, Ying Li, Ahmed S Elshikha, Denis Titov, Brian Brenner, Lee Seifer, Hope Pan, Nurdina Karic, Mohammad A Akbar, Yuanqing Lu, Sihong Song, Lei Zhou
Inflammaging plays an important role in most age-related diseases. However, the mechanism of inflammaging is largely unknown, and therapeutic control of inflammaging is challenging. Human alpha-1 antitrypsin (hAAT) has immune-regulatory, anti-inflammatory, and cytoprotective properties as demonstrated in several disease models including type 1 diabetes, arthritis, lupus, osteoporosis, and stroke. To test the potential anti-inflammaging effect of hAAT, we generated transgenic Drosophila lines expressing hAAT...
October 17, 2017: Aging Cell
https://www.readbyqxmd.com/read/29043847/smoking-related-interstitial-fibrosis-evidence-of-radiologic-regression-with-advancing-age-and-smoking-cessation
#2
Aurélie Fabre, Ann Treacy, Lisa P Lavelle, Maciek Narski, Nazia Faheem, David Healy, Jonathan D Dodd, Michael P Keane, James J Egan, Gilles Jebrak, Hervé Mal, Marcus W Butler
More data are needed regarding the radiology, co-morbidities and natural history of smoking-related interstitial fibrosis (SRIF), a common pathological finding, mainly described heretofore in association with lung cancer, where respiratory bronchiolitis (RB) usually co-exists. We prospectively acquired high resolution CT scan data (edge-enhancing lung reconstructions) to detect any radiologic interstitial lung abnormality (ILA) in individuals who ultimately underwent surgical lobectomy for lung cancer (n = 20), for radiologic/pathologic correlation...
October 18, 2017: COPD
https://www.readbyqxmd.com/read/29042303/changes-in-serum-proteins-in-dogs-with-ehrlichia-canis-infection
#3
Damián Escribano, Hüseyin Cihan, Silvia Martínez-Subiela, Pinar Levent, Meric Kocaturk, Nilüfer Aytug, Jose Joaquin Cerón, Asta Tvarijonaviciute, Zeki Yilmaz
The aim of this study was the identification of proteins differentially represented in the serum proteome of seropositive dogs with (Group 1) and without (Group 2) clinical-pathologic signs consistent with ehrlichiosis compared to healthy dogs. Serum samples were collected from 20 dogs of various breeds with naturally occurring ehrlichiosis (10 dogs belonged to Group 1 and 10 to Group 2) and 10 healthy dogs. Two-dimensional electrophoresis (2DE) of pooled serum for each of tge group of dogs were run in triplicate...
October 14, 2017: Microbial Pathogenesis
https://www.readbyqxmd.com/read/29039463/hepatic-differentiation-of-human-ipscs-in-different-3d-models-a-comparative-study
#4
Florian Meier, Nora Freyer, Joanna Brzeszczynska, Fanny Knöspel, Lyle Armstrong, Majlinda Lako, Selina Greuel, Georg Damm, Eva Ludwig-Schwellinger, Ulrich Deschl, James A Ross, Mario Beilmann, Katrin Zeilinger
Human induced pluripotent stem cells (hiPSCs) are a promising source from which to derive distinct somatic cell types for in vitro or clinical use. Existent protocols for hepatic differentiation of hiPSCs are primarily based on 2D cultivation of the cells. In the present study, the authors investigated the generation of hiPSC-derived hepatocyte-like cells using two different 3D culture systems: A 3D scaffold-free microspheroid culture system and a 3D hollow-fiber perfusion bioreactor. The differentiation outcome in these 3D systems was compared with that in conventional 2D cultures, using primary human hepatocytes as a control...
October 16, 2017: International Journal of Molecular Medicine
https://www.readbyqxmd.com/read/29034613/massive-ascites-and-the-heterozygous-alpha-1-antitrypsin-%C3%AE-1-at-living-related-donor-liver-in-the-homozygous-child
#5
Shirin E Khorsandi, Richard Thompson, Hector Vilca-Melendez, Anil Dhawan, Nigel Heaton
The following is a short report on the use of a heterozygous (PiMZ) alpha 1 antitrypsin (α1AT) living related donor liver in a homozygous (PiZ) child that was complicated by massive ascites early after transplant. This clinical report is then followed by a brief summary of present knowledge on the α1 AT protein and management of massive ascites in the pediatric liver transplant recipient.
October 15, 2017: Pediatric Transplantation
https://www.readbyqxmd.com/read/29033425/evaluation-by-mr-enterocolonography-of-lansoprazole-induced-collagenous-colitis-accompanied-with-protein-losing-enteropathy-a-case-report
#6
Akinori Mizoguchi, Masaaki Higashiyama, Keisuke Ikeyama, Shin Nishii, Hisato Terada, Hirotaka Furuhashi, Takeshi Takajo, Koji Maruta, Yuichi Yasutake, Kazuhiko Shirakabe, Chikako Watanabe, Kengo Tomita, Shunsuke Komoto, Shigeaki Nagao, Soichiro Miura, Ryota Hokari
We herein describe a 69-year-old man suffering from chronic diarrhea caused by lansoprazole (LPZ)-induced collagenous colitis (CC) accompanied with protein-losing enteropathy (PLE), diagnosed by increased fecal alpha-1 antitrypsin clearance and the findings of leakage from the descending colon to the sigmoid colon on scintigraphy. MR enterocolonography (MREC) was also performed for differentiating digestive diseases, and inflamed findings were observed around the same portion as those on scintigraphy, suggesting that this region was responsible for protein loss in this case...
October 16, 2017: Internal Medicine
https://www.readbyqxmd.com/read/29032169/survival-advantage-of-both-human-hepatocyte-xenografts-and-genome-edited-hepatocytes-for-treatment-of-%C3%AE-1-antitrypsin-deficiency
#7
Florie Borel, Qiushi Tang, Gwladys Gernoux, Cynthia Greer, Ziqiong Wang, Adi Barzel, Mark A Kay, Leonard D Shultz, Dale L Greiner, Terence R Flotte, Michael A Brehm, Christian Mueller
Hepatocytes represent an important target for gene therapy and editing of single-gene disorders. In α-1 antitrypsin (AAT) deficiency, one missense mutation results in impaired secretion of AAT. In most patients, lung damage occurs due to a lack of AAT-mediated protection of lung elastin from neutrophil elastase. In some patients, accumulation of misfolded PiZ mutant AAT protein triggers hepatocyte injury, leading to inflammation and cirrhosis. We hypothesized that correcting the Z mutant defect in hepatocytes would confer a selective advantage for repopulation of hepatocytes within an intact liver...
September 25, 2017: Molecular Therapy: the Journal of the American Society of Gene Therapy
https://www.readbyqxmd.com/read/29029865/total-proteolytic-activity-and-concentration-of-alpha-1-antitrypsin-in-meconium-for-assessment-of-the-protease-antiprotease-balance
#8
Ewa Skarżyńska, Bartosz Kiersztyn, Paulina Wilczyńska, Artur Jakimiuk, Barbara Lisowska-Myjak
BACKGROUND: During intrauterine life, various proteolytic enzymes and their main inhibitor, alpha-1 antitrypsin, accumulate naturally in meconium. A protease/antiprotease balance is required to maintain the biological stability of the environment in which the fetus develops. METHODS: The pool of active proteases was determined using the EnzChek Protease Assay Kit. The concentration of alpha-1 antitrypsin in meconium was measured by enzyme-linked immunosorbent assay...
October 4, 2017: European Journal of Obstetrics, Gynecology, and Reproductive Biology
https://www.readbyqxmd.com/read/28987255/genetic-testing-in-liver-disease-what-to-order-in-whom-and-when
#9
REVIEW
Emily A Schonfeld, Robert S Brown
Genetic causes of liver disease lead to a wide range of presentations, from mildly abnormal liver tests to acute liver failure. This article discusses the indications for testing and what to test for hereditary hemochromatosis, progressive familial intrahepatic cholestasis, benign recurrent intrahepatic cholestasis, lysosomal acid lipase deficiency, Gilbert syndrome, alpha-1 antitrypsin deficiency, and Wilson disease.
November 2017: Clinics in Liver Disease
https://www.readbyqxmd.com/read/28987047/-epidemiology-and-risk-factors-of-hepatocellular-carcinoma
#10
REVIEW
Agnieszka Budny, Piotr Kozłowski, Marzena Kamińska, Małgorzata Jankiewicz, Agnieszka Kolak, Bożena Budny, Witold Budny, Joanna Niemunis-Sawicka, Grzegorz Szczypiór, Bartosz Kurniawka, Franciszek Burdan
Primary liver neoplasms occurs relatively rarely in Poland. The most frequently occurring type of cancer is hepatocellular carcinoma (HCC), which globally constitutes 7% of all the occurrences of cancer. The incidence increases with age and is the highest in patients around the age of 70. It also varies significantly depending on the geographic location. The main factors that cause HCC are infection of HBV and HCV, whose genome integrates into the DNA of the host, causing mutations. The other factors include excessive alcohol consumption, contact or consumption of Aspergillus toxins as well as various metabolic disorders, such as α1-antitrypsin deficiency, hemochromatosis, tyrosinemia, porphyria, von Gierke disease and in person with gene mutation p...
September 29, 2017: Polski Merkuriusz Lekarski: Organ Polskiego Towarzystwa Lekarskiego
https://www.readbyqxmd.com/read/28985237/enterovirus-71-protease-2apro-and-3cpro-differentially-inhibit-the-cellular-endoplasmic-reticulum-associated-degradation-erad-pathway-via-distinct-mechanisms-and-enterovirus-71-hijacks-erad-component-p97-to-promote-its-replication
#11
Tao Wang, Bei Wang, He Huang, Chongyang Zhang, Yuanmei Zhu, Bin Pei, Chaofei Cheng, Lei Sun, Jianwei Wang, Qi Jin, Zhendong Zhao
Endoplasmic reticulum-associated degradation (ERAD) is an important function for cellular homeostasis. The mechanism of how picornavirus infection interferes with ERAD remains unclear. In this study, we demonstrated that enterovirus 71 (EV71) infection significantly inhibits cellular ERAD by targeting multiple key ERAD molecules with its proteases 2Apro and 3Cpro using different mechanisms. Ubc6e was identified as the key E2 ubiquitin-conjugating enzyme in EV71 disturbed ERAD. EV71 3Cpro cleaves Ubc6e at Q219G, Q260S, and Q273G...
October 6, 2017: PLoS Pathogens
https://www.readbyqxmd.com/read/28985109/bioequivalence-of-a-liquid-formulation-of-alpha1-proteinase-inhibitor-compared-with-prolastin%C3%A2-c-lyophilized-alpha1-pi-in-alpha1-antitrypsin-deficiency
#12
Alan F Barker, Michael A Campos, Mark L Brantly, James M Stocks, Robert A Sandhaus, Douglas Lee, Kimberly Steinmann, Jiang Lin, Susan Sorrells
This study evaluated the bioequivalence, safety, and immunogenicity of a new liquid formulation of human plasma-derived alpha1-proteinase inhibitor, Liquid Alpha1-PI, compared with the Lyophilized Alpha1-PI formulation (Prolastin®-C), for augmentation therapy in patients with alpha1-antitrypsin deficiency (AATD). In this double-blind, randomized, 20-week crossover study, 32 subjects with AATD were randomized to receive 8 weekly infusions of 60 mg/kg of Liquid Alpha1-PI or Lyophilized Alpha1-PI. Serial blood samples were drawn for 7 days after the last dose followed by 8 weeks of the alternative treatment...
October 6, 2017: COPD
https://www.readbyqxmd.com/read/28975833/the-role-of-diagnosis-and-treatment-of-underlying-liver-disease-for-the-prognosis-of-primary-liver-cancer
#13
Ashok Shiani, Shreya Narayanan, Luis Pena, Mark Friedman
Hepatocellular carcinoma (HCC) is the third leading cause of cancer-related deaths worldwide. Underlying chronic liver disease has been associated with an increased risk of developing HCC. This study is a review of the current literature regarding the diagnosis, prognostic significance, and role of treating underlying liver disease in patients who are at risk of primary liver cancer. Relevant peer review of the English literature between 1980 and 2017 within PubMed and the Cochrane library was conducted for scientific content on current advances in managing chronic liver diseases and the development of hepatocellular carcinoma...
July 2017: Cancer Control: Journal of the Moffitt Cancer Center
https://www.readbyqxmd.com/read/28974776/associations-between-snps-and-immune-related-circulating-proteins-in-schizophrenia
#14
Man K Chan, Jason D Cooper, Stefanie Heilmann-Heimbach, Josef Frank, Stephanie H Witt, Markus M Nöthen, Johann Steiner, Marcella Rietschel, Sabine Bahn
Genome-wide association studies (GWAS) and proteomic studies have provided convincing evidence implicating alterations in immune/inflammatory processes in schizophrenia. However, despite the convergence of evidence, direct links between the genetic and proteomic findings are still lacking for schizophrenia. We investigated associations between single nucleotide polymorphisms (SNPs) from the custom-made PsychArray and the expression levels of 190 multiplex immunoassay profiled serum proteins in 149 schizophrenia patients and 198 matched controls...
October 3, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28960647/long-term-fate-of-human-fetal-liver-progenitor-cells-transplanted-in-injured-mouse-livers
#15
Antony Irudayaswamy, Mark Muthiah, Lei Zhou, Hau Hung, Nur Halisah Bte Jumat, Jamil Haque, Narcissus Teoh, Geoffrey Farrell, Kimberly J Riehle, Jaymie Siqi Lin, Lin Lin Su, Jerry Ky Chan, Mahesh Choolani, Peng Cheang Wong, Aileen Wee, Seng Gee Lim, Jean Campbell, Nelson Fausto, Yock Young Dan
Liver progenitor cells have the potential to repair and regenerate a diseased liver. The success of any translational efforts, however, hinges on thorough understanding of the fate of these cells after transplant, especially in terms of long-term safety and efficacy. Here, we report transplantation of a liver progenitor population isolated from human fetal livers into immune-permissive mice with follow-up up to 36 weeks after transplant. We found that human progenitor cells engraft and differentiate into functional human hepatocytes in the mouse, producing albumin, alpha-1-antitrypsin, and glycogen...
September 28, 2017: Stem Cells
https://www.readbyqxmd.com/read/28947017/identification-of-a-new-defective-serpina1-allele-pi-zla-palma-encoding-an-alpha-1-antitrypsin-with-altered-glycosylation-pattern
#16
José M Hernández-Pérez, Ruth Ramos-Díaz, José A Pérez
BACKGROUND: Alpha-1-antitrypsin (AAT) deficiency is a genetic condition that arises from mutations in the SERPINA1 gene and predisposes to develop pulmonary emphysema and, less frequently, liver disease. Occasionally, new defective SERPINA1 alleles are detected as an outcome of targeted-screening programs or case-findings. METHODS: This study began with a female patient showing bronchial hyperreactivity. Serum level and phenotype for AAT was analysed by immunonephelometry and isoelectric focusing electrophoresis...
October 2017: Respiratory Medicine
https://www.readbyqxmd.com/read/28943279/lobar-emphysema-distribution-is-associated-with-5-year-radiological-disease-progression
#17
Adel Boueiz, Yale Chang, Michael H Cho, George R Washko, Raul San José Estépar, Russell P Bowler, James D Crapo, Dawn L DeMeo, Jennifer G Dy, Edwin K Silverman, Peter J Castaldi
BACKGROUND: Emphysema has considerable variability in its regional distribution. Craniocaudal emphysema distribution is an important predictor of the response to lung volume reduction. However, there is little consensus regarding how to define upper lobe-predominant and lower lobe-predominant emphysema subtypes. Consequently, the clinical and genetic associations with these subtypes are poorly characterized. METHODS: We sought to identify subgroups characterized by upper-lobe or lower-lobe emphysema predominance and comparable amounts of total emphysema by analyzing data from 9,210 smokers with non-alpha-1-antitrypsin deficiency in the Genetic Epidemiology of COPD (COPDGene) cohort...
September 21, 2017: Chest
https://www.readbyqxmd.com/read/28929906/the-important-role-of-primary-care-providers-in-the-detection-of-alpha-1-antitrypsin-deficiency
#18
Jorge E Lascano, Michael A Campos
OBJECTIVE: Alpha-1 antitrypsin deficiency (AATD) is an underrecognized genetic disorder that can cause chronic obstructive pulmonary disease (COPD) and liver cirrhosis, two clinical conditions commonly seen by primary care physicians. AATD is estimated to affect 1/4000-1/5000 people in the United States and 1-2% of all COPD cases. METHODS: PubMed was searched for relevant articles using AAT/AATD-related terms. RESULTS: Unfortunately, <10% of symptomatic individuals have been properly diagnosed primarily due to the underdiagnosis of COPD and the lack of awareness of AATD as a possible underlying cause...
October 5, 2017: Postgraduate Medicine
https://www.readbyqxmd.com/read/28927525/alpha-1-antitrypsin-deficiency-genetic-variations-clinical-manifestations-and-therapeutic-interventions
#19
REVIEW
Younis Mohammad Hazari, Arif Bashir, Mudasir Habib, Samirul Bashir, Huma Habib, M Abul Qasim, Naveed Nazir Shah, Ehtishamul Haq, Jeffrey Teckman, Khalid Majid Fazili
Alpha-1-antitrypsin (AAT) is an acute phase secretory glycoprotein that inhibits neutrophil proteases like elastase and is considered as the archetype of a family of structurally related serine-protease inhibitors termed serpins. Serum AAT predominantly originates from liver and increases three to five fold during host response to tissue injury and inflammation. The AAT deficiency is unique among the protein-misfolding diseases in that it causes target organ injury by both loss-of-function and gain-of-toxic function mechanisms...
July 2017: Mutation Research
https://www.readbyqxmd.com/read/28922398/role-of-the-p2-residue-of-human-alpha-1-antitrypsin-in-determining-target-protease-specificity
#20
Hye-Shin Chung, Ji-Sun Kim, Sang Mee Lee, Soon Jae Park
Alpha 1-antitrypsin (A1AT) is a serine protease inhibitor that mainly inhibits neutrophil elastase in the lungs. A variant of A1AT at the P1 position with methionine 358 to arginine (A1AT-Pittsburgh) is a rapid inhibitor of thrombin with greatly diminished anti-elastase activity. The P2 residue (position 357) of A1AT-Pittsburgh has been shown to play an important role in interactions with thrombin and kallikrein, but the role of P2 residue in wild-type A1AT has largely been unraveled. Here, we investigated the effects of P2 proline substitutions in wild-type A1AT on interactions with porcine pancreatic elastase (PPE) and human neutrophil elastase (HNE)...
2017: PloS One
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