Published in J Immunol on May 01, 2004
A surfactant protein C precursor protein BRICHOS domain mutation causes endoplasmic reticulum stress, proteasome dysfunction, and caspase 3 activation. Am J Respir Cell Mol Biol (2005) 1.90
Genetic modifiers of liver disease in cystic fibrosis. JAMA (2009) 1.88
Abnormal disulfide-linked oligomerization results in ER retention and altered signaling by TNFR1 mutants in TNFR1-associated periodic fever syndrome (TRAPS). Blood (2006) 1.61
Adaptation and increased susceptibility to infection associated with constitutive expression of misfolded SP-C. J Cell Biol (2006) 1.41
α-1 Antitrypsin regulates human neutrophil chemotaxis induced by soluble immune complexes and IL-8. J Clin Invest (2010) 1.38
GCN2 protein kinase is required to activate amino acid deprivation responses in mice treated with the anti-cancer agent L-asparaginase. J Biol Chem (2009) 1.29
NF-kappaB regulation: the nuclear response. J Cell Mol Med (2009) 1.27
Gene transfer of master autophagy regulator TFEB results in clearance of toxic protein and correction of hepatic disease in alpha-1-anti-trypsin deficiency. EMBO Mol Med (2013) 1.22
The intracellular accumulation of polymeric neuroserpin explains the severity of the dementia FENIB. Hum Mol Genet (2008) 1.20
Endoplasmic reticulum-associated degradation (ERAD) and autophagy cooperate to degrade polymerogenic mutant serpins. J Biol Chem (2009) 1.14
Polymers and inflammation: disease mechanisms of the serpinopathies. J Exp Med (2008) 1.06
Gene therapy for alpha-1 antitrypsin deficiency. Hum Mol Genet (2011) 1.04
Selenoprotein S/SEPS1 modifies endoplasmic reticulum stress in Z variant alpha1-antitrypsin deficiency. J Biol Chem (2009) 1.01
Alpha-1-antitrypsin deficiency: current concepts. Lung (2007) 1.00
Neuroserpin polymers activate NF-kappaB by a calcium signaling pathway that is independent of the unfolded protein response. J Biol Chem (2009) 1.00
Potential for therapeutic manipulation of the UPR in disease. Semin Immunopathol (2013) 0.99
The mammalian UPR boosts glycoprotein ERAD by suppressing the proteolytic downregulation of ER mannosidase I. J Cell Sci (2009) 0.98
Alpha-1 antitrypsin deficiency: a conformational disease associated with lung and liver manifestations. J Inherit Metab Dis (2008) 0.95
Rapamycin reduces intrahepatic alpha-1-antitrypsin mutant Z protein polymers and liver injury in a mouse model. Exp Biol Med (Maywood) (2010) 0.93
Why has it been so difficult to prove the efficacy of alpha-1-antitrypsin replacement therapy? Insights from the study of disease pathogenesis. Drug Des Devel Ther (2011) 0.92
The unfolded protein response in fatty liver disease. Semin Liver Dis (2013) 0.91
Protein Disulfide Isomerase Superfamily in Disease and the Regulation of Apoptosis. Endoplasmic Reticulum Stress Dis (2014) 0.89
Targeted protein destabilization reveals an estrogen-mediated ER stress response. Nat Chem Biol (2014) 0.89
MicroRNAs and liver cancer associated with iron overload: therapeutic targets unravelled. World J Gastroenterol (2013) 0.88
Expression of hereditary hemochromatosis C282Y HFE protein in HEK293 cells activates specific endoplasmic reticulum stress responses. BMC Cell Biol (2007) 0.86
Alpha1-antitrypsin deficiency: current perspective on research, diagnosis, and management. Int J Chron Obstruct Pulmon Dis (2006) 0.85
Z α-1 antitrypsin deficiency and the endoplasmic reticulum stress response. World J Gastrointest Pharmacol Ther (2010) 0.83
Cytosolic, autocrine alpha-1 proteinase inhibitor (A1PI) inhibits caspase-1 and blocks IL-1β dependent cytokine release in monocytes. PLoS One (2012) 0.83
Protein misfolding and the serpinopathies. Prion (2007) 0.83
Hepatic progenitor cell proliferation and liver injury in α-1-antitrypsin deficiency. J Pediatr Gastroenterol Nutr (2010) 0.82
Emergence of a stage-dependent human liver disease signature with directed differentiation of alpha-1 antitrypsin-deficient iPS cells. Stem Cell Reports (2015) 0.81
A novel interaction between aging and ER overload in a protein conformational dementia. Genetics (2013) 0.81
Inhibition of ICMT induces endothelial cell apoptosis through GRP94. Am J Respir Cell Mol Biol (2007) 0.81
Intracellular processing of alpha1-antitrypsin. Proc Am Thorac Soc (2010) 0.80
Increased ERK signalling promotes inflammatory signalling in primary airway epithelial cells expressing Z α1-antitrypsin. Hum Mol Genet (2013) 0.78
Characterising the association of latency with α(1)-antitrypsin polymerisation using a novel monoclonal antibody. Int J Biochem Cell Biol (2014) 0.78
Flotillin-1 facilitates toll-like receptor 3 signaling in human endothelial cells. Basic Res Cardiol (2014) 0.77
Modulation of the unfolded protein response during hepatocyte and cardiomyocyte apoptosis in trauma/hemorrhagic shock. Sci Rep (2013) 0.77
Interactions between N-linked glycosylation and polymerisation of neuroserpin within the endoplasmic reticulum. FEBS J (2015) 0.77
Role of Drosophila EDEMs in the degradation of the alpha-1-antitrypsin Z variant. Int J Mol Med (2015) 0.76
Multilineage transduction of resident lung cells in vivo by AAV2/8 for α1-antitrypsin gene therapy. Mol Ther Methods Clin Dev (2016) 0.75
FBG1 Is the Final Arbitrator of A1AT-Z Degradation. PLoS One (2015) 0.75
The endoplasmic reticulum remains functionally connected by vesicular transport after its fragmentation in cells expressing Z-α1-antitrypsin. FASEB J (2016) 0.75
The role of bronchial epithelial cells in the pathogenesis of COPD in Z-alpha-1 antitrypsin deficiency. Respir Res (2014) 0.75
Identification of eQTLs for hepatic Xbp1s and Socs3 gene expression in mice fed a high-fat, high-caloric diet. G3 (Bethesda) (2015) 0.75
Is there a therapeutic role for selenium in alpha-1 antitrypsin deficiency? Nutrients (2013) 0.75
Phase 2 clinical trial of a recombinant adeno-associated viral vector expressing α1-antitrypsin: interim results. Hum Gene Ther (2011) 2.75
Simvastatin decreases lipopolysaccharide-induced pulmonary inflammation in healthy volunteers. Am J Respir Crit Care Med (2009) 2.52
Nebulized hypertonic saline decreases IL-8 in sputum of patients with cystic fibrosis. Am J Respir Crit Care Med (2011) 2.37
Clarification of the risk of chronic obstructive pulmonary disease in α1-antitrypsin deficiency PiMZ heterozygotes. Am J Respir Crit Care Med (2014) 2.14
Effect of estrogen on pseudomonas mucoidy and exacerbations in cystic fibrosis. N Engl J Med (2012) 1.93
Z alpha1-antitrypsin polymerizes in the lung and acts as a neutrophil chemoattractant. Chest (2004) 1.88
Anti-proline-glycine-proline or antielastin autoantibodies are not evident in chronic inflammatory lung disease. Am J Respir Crit Care Med (2009) 1.86
TLR-induced inflammation in cystic fibrosis and non-cystic fibrosis airway epithelial cells. J Immunol (2005) 1.67
Secretory leucoprotease inhibitor binds to NF-kappaB binding sites in monocytes and inhibits p65 binding. J Exp Med (2005) 1.65
Endotoxin up-regulates interleukin-18: potential role for gram-negative colonization in sarcoidosis. Am J Respir Crit Care Med (2005) 1.57
Secretory leucoprotease inhibitor prevents lipopolysaccharide-induced IkappaBalpha degradation without affecting phosphorylation or ubiquitination. J Biol Chem (2002) 1.52
Alpha-1 antitrypsin deficiency. Respir Med (2010) 1.51
Granzyme B-dependent proteolysis acts as a switch to enhance the proinflammatory activity of IL-1α. Mol Cell (2011) 1.48
miR-199a-5p silencing regulates the unfolded protein response in chronic obstructive pulmonary disease and α1-antitrypsin deficiency. Am J Respir Crit Care Med (2014) 1.46
α1-Antitrypsin activates protein phosphatase 2A to counter lung inflammatory responses. Am J Respir Crit Care Med (2014) 1.45
miR-31 dysregulation in cystic fibrosis airways contributes to increased pulmonary cathepsin S production. Am J Respir Crit Care Med (2014) 1.45
Extubation versus tracheostomy in withdrawal of treatment-ethical, clinical, and legal perspectives. J Crit Care (2009) 1.44
Adult-onset nemaline myopathy presenting as respiratory failure. Respir Care (2008) 1.40
Neutrophil elastase up-regulates interleukin-8 via toll-like receptor 4. FEBS Lett (2003) 1.38
α-1 Antitrypsin regulates human neutrophil chemotaxis induced by soluble immune complexes and IL-8. J Clin Invest (2010) 1.38
miR-126 is downregulated in cystic fibrosis airway epithelial cells and regulates TOM1 expression. J Immunol (2010) 1.38
Epithelial expression of TLR4 is modulated in COPD and by steroids, salmeterol and cigarette smoke. Respir Res (2007) 1.31
Loss of microbicidal activity and increased formation of biofilm due to decreased lactoferrin activity in patients with cystic fibrosis. J Infect Dis (2004) 1.25
MicroRNAs in inflammatory lung disease--master regulators or target practice? Respir Res (2010) 1.24
Inactivation of human beta-defensins 2 and 3 by elastolytic cathepsins. J Immunol (2003) 1.24
LL-37 complexation with glycosaminoglycans in cystic fibrosis lungs inhibits antimicrobial activity, which can be restored by hypertonic saline. J Immunol (2009) 1.22
Respiratory epithelial cells require Toll-like receptor 4 for induction of human beta-defensin 2 by lipopolysaccharide. Respir Res (2005) 1.21
Activation of the epidermal growth factor receptor (EGFR) by a novel metalloprotease pathway. J Biol Chem (2008) 1.20
Human Treg responses allow sustained recombinant adeno-associated virus-mediated transgene expression. J Clin Invest (2013) 1.20
Secretory leucoprotease inhibitor impairs Toll-like receptor 2- and 4-mediated responses in monocytic cells. Infect Immun (2004) 1.20
Proteases and antiproteases in chronic neutrophilic lung disease - relevance to drug discovery. Br J Pharmacol (2009) 1.18
Antimicrobial proteins and polypeptides in pulmonary innate defence. Respir Res (2006) 1.18
Elastolytic proteases: inflammation resolution and dysregulation in chronic infective lung disease. Am J Respir Crit Care Med (2005) 1.18
Neutrophil elastase up-regulates cathepsin B and matrix metalloprotease-2 expression. J Immunol (2007) 1.17
SLPI and elafin: multifunctional antiproteases of the WFDC family. Biochem Soc Trans (2011) 1.12
Toll-like receptor expression and function in airway epithelial cells. Arch Immunol Ther Exp (Warsz) (2005) 1.09
Sputum Candida albicans presages FEV₁ decline and hospital-treated exacerbations in cystic fibrosis. Chest (2010) 1.09
17Beta-estradiol inhibits IL-8 in cystic fibrosis by up-regulating secretory leucoprotease inhibitor. Am J Respir Crit Care Med (2010) 1.08
Decreased levels of secretory leucoprotease inhibitor in the Pseudomonas-infected cystic fibrosis lung are due to neutrophil elastase degradation. J Immunol (2009) 1.07
Cytosolic pH and the inflammatory microenvironment modulate cell death in human neutrophils after phagocytosis. Blood (2002) 1.06
Evidence for unfolded protein response activation in monocytes from individuals with alpha-1 antitrypsin deficiency. J Immunol (2010) 1.05
Prevalence, risk factors and impact on daytime sleepiness and hypertension of periodic leg movements with arousals in patients with obstructive sleep apnea. J Clin Sleep Med (2006) 1.03
Depression and sleep-related symptoms in an adult, indigenous, North American population. J Clin Sleep Med (2008) 1.03
Elafin, an elastase-specific inhibitor, is cleaved by its cognate enzyme neutrophil elastase in sputum from individuals with cystic fibrosis. J Biol Chem (2008) 1.03