Measuring the Effect of Histone Deacetylase Inhibitors (HDACi) on the Secretion and Activity of Alpha-1 Antitrypsin.
Sign Up to like & getrecommendations! Published in 2017 at "Methods in molecular biology"
DOI: 10.1007/978-1-4939-7163-3_18
Abstract: Alpha-1 antitrypsin deficiency (AATD) is a protein conformational disease with the most common cause being the Z-variant mutation in alpha-1 antitrypsin (Z-AAT). The misfolded conformation triggered by the Z-variant disrupts cellular proteostasis (protein folding) systems… read more here.
Keywords: secretion; antitrypsin; alpha antitrypsin; histone deacetylase ... See more keywords
Alpha‐1‐antitrypsin ameliorates inflammation and neurodegeneration in the diabetic mouse retina
Sign Up to like & getrecommendations! Published in 2018 at "Experimental Eye Research"
DOI: 10.1016/j.exer.2018.05.013
Abstract: ABSTRACT Diabetic retinopathy (DR) is the most common cause of blindness in the working age population. Early events of DR are accompanied by neurodegeneration of the inner retina resulting in ganglion cell loss. These findings… read more here.
Keywords: diabetic mouse; aat treated; antitrypsin; alpha antitrypsin ... See more keywords
Alpha1 antitrypsin deficiency due to an homozygous PI* Null Q0Cairo mutation: Early onset of pulmonary manifestations and variability of clinical expression
Sign Up to like & getrecommendations! Published in 2018 at "Respiratory Medicine Case Reports"
DOI: 10.1016/j.rmcr.2018.04.005
Abstract: Alpha-1 antitrypsin deficiency is an autosomal, codominant disorder caused by mutations of the SERPINA1 gene. This genetic disorder is mainly associated with development of pulmonary emphysema and/or chronic liver disease and cirrhosis. Here we report… read more here.
Keywords: null q0cairo; antitrypsin; alpha antitrypsin; antitrypsin deficiency ... See more keywords
Safety of Intravenous Administration of an AAV8 Vector Coding for an Oxidation Resistant Human α1-antitrypsin for the Treatment of α1-antitrypsin Deficiency.
Sign Up to like & getrecommendations! Published in 2023 at "Human gene therapy"
DOI: 10.1089/hum.2022.192
Abstract: α1-antitrypsin (AAT) deficiency is a common autosomal recessive hereditary disorder, with a high risk for the development of early onset panacinar emphysema. AAT produced primarily in the liver, functions to protect the lung from neutrophil… read more here.
Keywords: aav8haat avl; antitrypsin; intravenous administration; study ... See more keywords
Untangling Lower Airway Dysbiosis in Critically Ill Patients with COVID-19
Sign Up to like & getrecommendations! Published in 2022 at "American Journal of Respiratory and Critical Care Medicine"
DOI: 10.1164/rccm.202206-1074ed
Abstract: Gynecol 2021;138:494–506. 7. Miravitlles M, Dirksen A, Ferrarotti I, Koblizek V, Lange P, Mahadeva R, et al. European Respiratory Society statement: diagnosis and treatment of pulmonary disease in a1-antitrypsin deficiency. Eur Respir J 2017;50: 1700610.… read more here.
Keywords: radiology; respir; deficiency; antitrypsin ... See more keywords
Design and characterization of α1-antitrypsin variants for treatment of contact system-driven thromboinflammation.
Sign Up to like & getrecommendations! Published in 2019 at "Blood"
DOI: 10.1182/blood.2019000481
Abstract: The contact system produces the inflammatory peptide bradykinin and contributes to experimental thrombosis. C1 esterase-inhibitor (C1INH) deficiency or gain-of-function mutations in Factor XII (FXII) cause hereditary angioedema, a life-threatening tissue swelling disease. C1INH is a… read more here.
Keywords: system; system driven; antitrypsin; treatment contact ... See more keywords
A transgenic zebrafish model of hepatocyte function in human Z α1-antitrypsin deficiency
Sign Up to like & getrecommendations! Published in 2019 at "Biological Chemistry"
DOI: 10.1515/hsz-2018-0391
Abstract: Abstract In human α1-antitrypsin deficiency, homozygous carriers of the Z (E324K) mutation in the gene SERPINA1 have insufficient circulating α1-antitrypsin and are predisposed to emphysema. Misfolding and accumulation of the mutant protein in hepatocytes also… read more here.
Keywords: human antitrypsin; antitrypsin; zebrafish model; antitrypsin deficiency ... See more keywords
S-Nitrosylation of α1-Antitrypsin Triggers Macrophages Toward Inflammatory Phenotype and Enhances Intra-Cellular Bacteria Elimination
Sign Up to like & getrecommendations! Published in 2019 at "Frontiers in Immunology"
DOI: 10.3389/fimmu.2019.00590
Abstract: Background: Human α1-antitrypsin (hAAT) is a circulating anti-inflammatory serine-protease inhibitor that rises during acute phase responses. in vivo, hAAT reduces bacterial load, without directly inhibiting bacterial growth. In conditions of excess nitric-oxide (NO), hAAT undergoes… read more here.
Keywords: bacteria elimination; antitrypsin; nitrosylation; haat ... See more keywords
The Delivery of α1-Antitrypsin Therapy Through Transepidermal Route: Worthwhile to Explore
Sign Up to like & getrecommendations! Published in 2020 at "Frontiers in Pharmacology"
DOI: 10.3389/fphar.2020.00983
Abstract: Human α1-antitrypsin (AAT) is an abundant acute phase glycoprotein expressing anti-protease and immunomodulatory activities, and is used as a biopharmaceutical to treat patients with inherited AAT deficiency. The pleiotropic properties of AAT provide a rationale… read more here.
Keywords: worthwhile explore; aat; antitrypsin; transepidermal route ... See more keywords
Investigating the Link between Alpha-1 Antitrypsin and Human Neutrophil Elastase in Bronchoalveolar Lavage Fluid of COVID-19 Patients
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DOI: 10.3390/cimb44050143
Abstract: Neutrophils play a pathogenic role in COVID-19 by releasing Neutrophils Extracellular Traps (NETs) or human neutrophil elastase (HNE). Given that HNE is inhibited by α1-antitrypsin (AAT), we aimed to assess the content of HNE, α1-antitrypsin… read more here.
Keywords: hne; antitrypsin; neutrophil elastase; covid ... See more keywords
Accelerated Wound Border Closure Using a Microemulsion Containing Non-Inhibitory Recombinant α1-Antitrypsin
Sign Up to like & getrecommendations! Published in 2022 at "International Journal of Molecular Sciences"
DOI: 10.3390/ijms23137364
Abstract: Wound healing requires a non-compromising combination of inflammatory and anti-inflammatory processes. Human α1-antitrypsin (hAAT), a circulating glycoprotein that rises during acute-phase responses and during healthy pregnancies, is tissue-protective and tolerance-inducing; although anti-inflammatory, hAAT enhances revascularization.… read more here.
Keywords: microemulsion; wound healing; antitrypsin; haat ... See more keywords