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Model process for removal of caseins from milk of transgenic animals
BIOTECHNOLOGY PROGRESS
Expression and purification of functional human alpha-1-antitrypsin from cultured plant cells
BIOTECHNOLOGY PROGRESS
Analysis by matrix assisted laser desorption/ionisation-time of flight mass spectrometry of the post-translational modifications of alpha(1)-antitrypsin isoforms separated by two-dimensional polyacrylamide gel electrophoresis
PROTEOMICS
Frequency of nonalcoholic steatohepatitis as a cause of advanced liver disease
LIVER TRANSPLANTATION
Epstein-Barr virus/human vector provides high-level, long-term expression of alpha(1)-antitrypsin in mice
MOLECULAR THERAPY
Linear DNAs concatemerize in vivo and result in sustained transgene expression in mouse liver
MOLECULAR THERAPY
A linkage disequilibrium between genes at the serine protease inhibitor gene cluster on chromosome 14q32.1 is associated with Wegener's granulomatosis
CLINICAL IMMUNOLOGY
A novel ER alpha-mannosidase-like protein accelerates ER-associated degradation
EMBO REPORTS
The genetics of chronic obstructive pulmonary disease
RESPIRATORY RESEARCH
Osmolytes as modulators of conformational changes in serpins
BIOLOGICAL CHEMISTRY
Hepatocellular ubiquitin expression in alpha(1)-antitrypsin deficiency
PEDIATRIC AND DEVELOPMENTAL PATHOLOGY
In vivo complex formation of oxidized alpha(1)-antitrypsin and LDL
ARTERIOSCLEROSIS THROMBOSIS AND VASCULAR BIOLOGY
Effects of cigarette smoke in mice with different levels of alpha(1)-proteinase inhibitor and sensitivity to oxidants
AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE
Neutrophils, unopposed neutrophil elastase, and alpha(1)-antiprotease defenses following human lung transplantation
AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE
Self antigen prognostic for human immunodeficiency virus disease progression
CLINICAL AND DIAGNOSTIC LABORATORY IMMUNOLOGY
Mutation detection in the alpha-1 antitrypsin gene (PI) using denaturing gradient gel electrophoresis
HUMAN MUTATION
Therapeutic elastase inhibition by alpha-1-antitrypsin gene transfer limits neointima formation in normal rabbits
JOURNAL OF VASCULAR AND INTERVENTIONAL RADIOLOGY
Prevention of polymerization of M and Z alpha(1)-antitrypsin (alpha(1)-AT)with trimethylamine N-oxide - Implications for the treatment of alpha(1)-AT deficiency
AMERICAN JOURNAL OF RESPIRATORY CELL AND MOLECULAR BIOLOGY
Anti-proteinase 3 antibody activation of neutrophils can be inhibited by alpha 1-antitrypsin
AMERICAN JOURNAL OF RESPIRATORY CELL AND MOLECULAR BIOLOGY
CMV-beta-actin promoter directs higher expression from an adeno-associatedviral vector in the liver than the cytomegalovirus or elongation factor 1 alpha promoter and results in therapeutic levels of human factor X in mice
HUMAN GENE THERAPY
Genetic predisposition to alcoholic toxic cirrhosis
RUSSIAN JOURNAL OF GENETICS
An alpha(1)-antitrypsin enhancer polymorphism is a genetic modifier of pulmonary outcome in cystic fibrosis
EUROPEAN JOURNAL OF HUMAN GENETICS
Stable therapeutic serum levels of human alpha-1 antitrypsin (AAT) after portal vein injection of recombinant adeno-associated virus (rAAV) vectors
GENE THERAPY
Cavities of alpha(1)-antitrypsin that play structural and functional roles
PROTEIN SCIENCE
Extending the capabilities of targeted molecular dynamics: Simulation of alarge conformational transition in plasminogen activator inhibitor 1
PROTEIN SCIENCE
Increased concentration of fecal alpha(1)-antitrypsin is associated with cow's milk allergy in infants with atopic eczema
CLINICAL AND EXPERIMENTAL ALLERGY
Alpha-1-antitrypsin is present in the specific granules of human eosinophilic granulocytes
CLINICAL AND EXPERIMENTAL ALLERGY
Exhaled nitric oxide in patients with PiZZ Phenotype-related alpha 1-anti-trypsin deficiency
RESPIRATORY MEDICINE
alpha 1-antitrypsin Pi phenotypes S and Z in Spain: an analysis of the published surveys
RESPIRATORY MEDICINE
Transforming functions of Simian Virus 40
ONCOGENE
Asialofetuin liposome-mediated human alpha(1)-antitrypsin gene transfer invivo results in stationary long-term gene expression
JOURNAL OF MOLECULAR MEDICINE-JMM
Comparison of outcome after pediatric liver transplantation for metabolic diseases and biliary atresia
EUROPEAN JOURNAL OF PEDIATRIC SURGERY
Henoch-Schonlein purpura with IgG PR3-ANCA in a PiZZ alpha 1-antitrypsin deficient patient
NEPHROLOGY DIALYSIS TRANSPLANTATION
High proteinuria selectivity index based upon IgM is a strong predictor ofpoor renal survival in glomerular diseases
NEPHROLOGY DIALYSIS TRANSPLANTATION
Analysis of anti-neutrophil cytoplasmic antibodies (ANCA): frequency and specificity in a sample of 191 homozygous (PiZZ) alpha1-antitrypsin-deficient subjects
NEPHROLOGY DIALYSIS TRANSPLANTATION
Conformational properties of serine proteinase inhibitors (serpins) confermultiple pathophysiological roles
BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE
Compound heterozygosity for alpha-1-antitrypsin (S-iiyama and QO(clayton))in an oriental patient
INTERNAL MEDICINE
Adenomatoid odontogenic tumour: immunohistochemical demonstration of transferrin, ferritin and alpha-one-antitrypsin
JOURNAL OF ORAL PATHOLOGY & MEDICINE
High-resolution computed tomography scanning in alpha(1)-antitrypsin deficiency: relationship to lung function and health status
EUROPEAN RESPIRATORY JOURNAL
Increased circulating levels of alpha(1)-antitrypsin and calprotectin are associated with reduced gas diffusion in the lungs
EUROPEAN RESPIRATORY JOURNAL
Epidemiology of chronic obstructive pulmonary disease
EUROPEAN RESPIRATORY JOURNAL
Alpha-1-antitrypsin genotyping with mouthwash specimens
EUROPEAN RESPIRATORY JOURNAL
Rare alpha-1-antitrypsin phenotypes and liver-test abnormalities during infancy
ACTA PAEDIATRICA
Proteinase 3, Wegener's autoantigen: from gene to antigen
JOURNAL OF LEUKOCYTE BIOLOGY
Phylogenetic analyses of amino acid variation in the serpin proteins
MOLECULAR BIOLOGY AND EVOLUTION
Vertebrate serpins: Construction of a conflict-free phylogeny by combiningexon-intron and diagnostic site analyses
MOLECULAR BIOLOGY AND EVOLUTION
Alpha 1-antitrypsin deficiency-associate panniculitis
MEDIZINISCHE KLINIK
Characterization of C-terminal peptide of recombinant protein by use of anti-peptide antibody
KAGAKU KOGAKU RONBUNSHU
Chromosomal sequences flanking an efficiently expressed transgene dramatically enhance its expression
GENE
Patterns of haplotype diversity within the serpin gene cluster at 14q32.1:insights into the natural history of the alpha 1-antitrypsin polymorphism
HUMAN GENETICS
The conformational basis of thrombosis
THROMBOSIS AND HAEMOSTASIS
Fibrillogenic C-terminal fragment of alpha-1-antitrypsin activates human monocytes via oxidative mechanisms
CELL AND TISSUE RESEARCH
Comparison between the thermodynamic features of alpha 1-antitrypsin and human albumin partitioning in aqueous two-phase systems of polyethyleneglycol-dextran
BIOPHYSICAL CHEMISTRY
Serum alpha-1-antitrypsin concentration during normal and diabetic pregnancy
EUROPEAN JOURNAL OF OBSTETRICS GYNECOLOGY AND REPRODUCTIVE BIOLOGY
Observations of an idiopathic granulomatous mastitis
EUROPEAN JOURNAL OF OBSTETRICS GYNECOLOGY AND REPRODUCTIVE BIOLOGY
alpha(1)-antitrypin deficiency-associated liver disease in Turkish children
JOURNAL OF PEDIATRIC GASTROENTEROLOGY AND NUTRITION
Congenital disorders of glycosylation type I leads to altered processing of N-linked glycans, as well as underglycosylation
BIOCHEMICAL JOURNAL
Synthesis of hepatic secretory proteins in normal adults consuming a diet marginally adequate in protein
AMERICAN JOURNAL OF PHYSIOLOGY-GASTROINTESTINAL AND LIVER PHYSIOLOGY
Panniculitis. Part 11. Mostly lobular panniculitis
JOURNAL OF THE AMERICAN ACADEMY OF DERMATOLOGY
Electrophoretic characterization of heat-stable squamous cell carcinoma antigen
ELECTROPHORESIS
Pulmonary function tests, high-resolution computerized tomography, alpha(1)-antitrypsin measurement, and early detection of pulmonary involvement in patients with systemic sclerosis
RHEUMATOLOGY INTERNATIONAL
Intracellular location and nuclear targeting of the Spi-1, Spi-2 and Spi-3gene-derived serine protease inhibitors in non-secretory cells
EUROPEAN JOURNAL OF CELL BIOLOGY
New developments in affinity chromatography with potential application in the production of biopharmaceuticals
JOURNAL OF BIOCHEMICAL AND BIOPHYSICAL METHODS
The role of hepatocytes in the clearance of liposomes from the blood circulation
PROGRESS IN LIPID RESEARCH
Inhalation of [I-123]alpha(1)-protease inhibitor: Toward a new therapeuticconcept of alpha(1)-protease inhibitor deficiency?
JOURNAL OF NUCLEAR MEDICINE
Molecular analysis of the Pi*Z allele in patients with liver disease
AMERICAN JOURNAL OF MEDICAL GENETICS
Alagille syndrome associated with a paracentric inversion 20p12.2p13 disrupting the JAG1 gene
AMERICAN JOURNAL OF MEDICAL GENETICS
Pilot detection study of alpha(1) antitrypsin deficiency in a targeted population
AMERICAN JOURNAL OF MEDICAL GENETICS
Different patterns of lymphoscintigraphic findings in patients with intestinal lymphangiectasia
NUCLEAR MEDICINE COMMUNICATIONS
Detection of early gene expression changes by differential display in the livers of mice exposed to dichloroacetic acid
CARCINOGENESIS
Production and recovery of recombinant protease inhibitor alpha(1)-antitrypsin
ENZYME AND MICROBIAL TECHNOLOGY
Research opportunities and advances in lung disease
JAMA-JOURNAL OF THE AMERICAN MEDICAL ASSOCIATION
Chromosome 14 linkage analysis and mutation study of 2 serpin genes in allergic asthmatic families
JOURNAL OF ALLERGY AND CLINICAL IMMUNOLOGY
Determination of alpha(1)-proteinase inhibitor by a new enzyme linked immunosorbant assay in feces, serum and an enterocyte-like cell line
ZEITSCHRIFT FUR GASTROENTEROLOGIE
Preparation and properties of an alpha-1-protease inhibitor concentrate with high specific activity
VOX SANGUINIS
Systemic anti-inflammatory mediators in COPD: increase in soluble interleukin 1 receptor II during treatment of exacerbations
THORAX
Animal models of chronic obstructive pulmonary disease
THORAX
Association of alpha(1)-antichymotrypsin deficiency with milder lung disease in patients with cystic fibrosis
THORAX
Factors affecting formation and growth of intracranial aneurysms - A long-term follow-up study
STROKE
Genetic and environmental modulation of chronic obstructive pulmonary disease
RESPIRATION PHYSIOLOGY
A case of protein-losing enteropathy caused by intestinal lymphangiectasiain a preterm infant
PEDIATRICS
Update on panniculitis
MOUNT SINAI JOURNAL OF MEDICINE
Association of cystic fibrosis, coeliac disease and alpha-1-antitrypsin deficiency in a child of 4 years
MONATSSCHRIFT KINDERHEILKUNDE
A 2.1 angstrom resolution structure of an uncleaved alpha(1)-antitrypsin shows variability of the reactive center and other loops
JOURNAL OF MOLECULAR BIOLOGY
alpha-1 Antitrypsin phenotypes by isoelectric focusing in a metropolitan southern Chinese population
JOURNAL OF CLINICAL PATHOLOGY
A common regulatory locus affects both HNF4/HNF1 alpha pathway activation and sensitivity to LPS-mediated apoptosis in rat hepatoma cells
JOURNAL OF CELL SCIENCE
Misfolded growth hormone causes fragmentation of the Golgi apparatus and disrupts endoplasmic reticulum-to-Golgi traffic
JOURNAL OF CELL SCIENCE
Crystal structure of the complex of plasminogen activator inhibitor 2 witha peptide mimicking the reactive center loop
JOURNAL OF BIOLOGICAL CHEMISTRY
A naturally occurring nonpolymerogenic mutant of alpha 1-antitrypsin characterized by prolonged retention in the endoplasmic reticulum
JOURNAL OF BIOLOGICAL CHEMISTRY
The serpin inhibitory mechanism is critically dependent on the length of the reactive center loop
JOURNAL OF BIOLOGICAL CHEMISTRY
Structural and energetic characteristics of the heparin-binding site in antithrombotic protein C
JOURNAL OF BIOLOGICAL CHEMISTRY
Prostasin is a glycosylphosphatidylinositol-anchored active serine protease
JOURNAL OF BIOLOGICAL CHEMISTRY
C-terminal fragment of alpha 1-antitrypsin activates human monocytes to a pro-inflammatory state through interactions with the CD36 scavenger receptor and LDL receptor
ATHEROSCLEROSIS
Mutations of the pancreatic secretory trypsin inhibitor (PSTI) gene in idiopathic chronic pancreatitis - Reply
GASTROENTEROLOGY
Crystal structure of neuroserpin: a neuronal serpin involved in a conformational disease
FEBS LETTERS
Polymorphism of the beta(2)-adrenoceptor in COPD in Chinese subjects
CHEST
Does reduced alpha(1)-antitrypsin activity explain the link between cigarette smoking and idiopathic pulmonary fibrosis?
CHEST
Longitudinal follow-up of patients with alpha(1)-protease inhibitor deficiency before and during therapy with IV alpha(1)-protease inhibitor
CHEST
The direct medical costs of alpha-antitrypsin deficiency
CHEST
Treatment of alpha(1)-antitrypsin-deficiency panniculitis with minocycline
CUTIS