Alpha(1)-antitrypsin deficiency, liver disease and emphysema.

No abstract is available for this paper.

• Publication year

  2003

• Venue

  International Journal of Biochemistry and Cell Biology

• Publication date

  Unknown publication date

• Fields of study

  Medicine

• Identifiers
  PMID 12672469
• External record

  Open on Semantic Scholar

• Source metadata

  Semantic Scholar, PubMed

• No claims are published for this paper.

• No concepts are published for this paper.

• Polymers of alpha(1)-antitrypsin are chemotactic for human neutrophils: a new paradigm for the pathogenesis of emphysema.

  2002cited by this paper
• 6-mer Peptide Selectively Anneals to a Pathogenic Serpin Conformation and Blocks Polymerization

  2002cited by this paper
• Alpha1-antitrypsin deficiency--a model for conformational diseases.

  2002cited by this paper
• Prevention of polymerization of M and Z alpha1-Antitrypsin (alpha1-AT) with trimethylamine N-oxide. Implications for the treatment of alpha1-at deficiency.

  2001cited by this paper
• The Proteasome Participates in Degradation of Mutant α1-Antitrypsin Z in the Endoplasmic Reticulum of Hepatoma-derived Hepatocytes*

  2001cited by this paper
• The Serpins Are an Expanding Superfamily of Structurally Similar but Functionally Diverse Proteins

  2001cited by this paper
• Processing by Endoplasmic Reticulum Mannosidases Partitions a Secretion-impaired Glycoprotein into Distinct Disposal Pathways*

  2000cited by this paper
• Chemical chaperones mediate increased secretion of mutant alpha 1-antitrypsin (alpha 1-AT) Z: A potential pharmacological strategy for prevention of liver injury and emphysema in alpha 1-AT deficiency.

  2000cited by this paper
• Heteropolymerization of S, I, and Z alpha1-antitrypsin and liver cirrhosis.

  1999cited by this paper
• Alpha 1-antitrypsin Siiyama (Ser53-->Phe). Further evidence for intracellular loop-sheet polymerization.

  1993cited by this paper
• The mechanism of Z alpha 1-antitrypsin accumulation in the liver.

  1992cited by this paper
• Lung lavage fluid from patients with alpha 1-proteinase inhibitor deficiency or chronic obstructive bronchitis: anti-elastase function and cell profile.

  1987cited by this paper
• EXPERIMENTAL EMPHYSEMA: ITS PRODUCTION WITH PAPAIN IN NORMAL AND SILICOTIC RATS.

  1965cited by this paper

• Liver Disease and Prevalence of Liver Transplantation in Adults With ZZ Alpha‐1 Antitrypsin Deficiency—A Meta‐Analysis

  2025cites this paper
• TissueMosaic: Self-supervised learning of tissue representations enables differential spatial transcriptomics across samples.

  2025cites this paper
• Efficacy and safety of the spiration valve System™ for treatment of severe emphysema in patients with Alpha-1 antitrypsin deficiency (EMPROVE).

  2024cites this paper
• Proteolysis and Deficiency of α1-Proteinase Inhibitor in SARS-CoV-2 Infection

  2022cites this paper
• Proteolysis and Deficiency of α1-Proteinase Inhibitor in SARS-CoV-2 Infection

  2022cites this paper
• SERPINA1 Gene

  2020cites this paper
• Alpha 1-Antitrypsin Inhibitor Therapy

  2019cites this paper
• Liver damage in a patient with Gaucher's disease type 1 and alpha-1 antitrypsin deficiency: a potential epigenetic effect?

  2019cites this paper
• rBmTI-6 attenuates pathophysiological and inflammatory parameters of induced emphysema in mice.

  2018cites this paper
• Genetic diagnosis of α1-antitrypsin deficiency using DNA from buccal swab and serum samples

  2017cites this paper
• Evaluation of Alpha-1 Antitrypsin Levels and SERPINA1 Gene Polymorphisms in Sickle Cell Disease

  2017cites this paper
• Liver Transplantation for Monogenic Metabolic Diseases Involving the Kidney

  2017cites this paper
• Alpha-1 antitrypsin: Associated diseases and therapeutic uses.

  2016cites this paper
• Role of Drosophila EDEMs in the degradation of the alpha-1-antitrypsin Z variant

  2015cites this paper
• The Role of Lectin-Carbohydrate Interactions in the Regulation of ER-Associated Protein Degradation

  2015cites this paper
• Micro-CT analysis of paraffin embedded lung tissue : is small airway obstruction an early feature of COPD?

  2014cites this paper
• Hereditary of Alpha-1-Antitrypsin Deficiency

  2013cites this paper
• Low Incidence of α-1 antitrypsin deficiency among babies with prolonged jaundice.

  2013cites this paper
• Monogenic diseases that can be cured by liver transplantation.

  2013cites this paper
• Fibromyalgia, mood disorders, and intense creative energy: A1AT polymorphisms are not always silent.

  2012cites this paper
• In vitro evaluation of the antielastase activity of polycyclic β-lactams.

  2012cites this paper
• Paediatric liver transplantation for metabolic disorders. Part 2: Metabolic disorders with liver lesions.

  2011cites this paper
• Role of the Lectin VIP36 in Post‐ER Quality Control of Human α1‐Antitrypsin

  2010cites this paper
• Alpha1-antitrypsin deficiency.

  2009cites this paper
• Automated determination of serum alpha1-antitrypsin by antitryptic activity measurement.

  2009cites this paper
• Effects of Sp1 overexpression on cultured human corneal stromal cells

  2009cites this paper
• Matrix metalloproteinase-9/gelatinase B is a putative therapeutic target of chronic obstructive pulmonary disease and multiple sclerosis.

  2008cites this paper
• Protein subcellular localization prediction using artificial intelligence technology.

  2008cites this paper
• Exploring functional and folding energy landscapes by hydrogen-deuterium exchange mass spectrometry.

  2008cites this paper
• Le rôle de CD81 et de ses protéines associées dans le mécanisme d'entrée du virus de l'hépatite C

  2008cites this paper
• ADD66, a gene involved in the endoplasmic reticulum-associated degradation of alpha-1-antitrypsin-Z in yeast, facilitates proteasome activity and assembly.

  2007cites this paper
• Status of clinical gene sequencing data reporting and associated risks for information loss

  2007cites this paper
• Art, alpha-1-antitrypsin polymorphisms and intense creative energy: blessing or curse?

  2007cites this paper
• How a cell deals with abnormal proteins. Pathogenetic mechanisms in protein aggregation diseases.

  2007cites this paper
• How a Cell Deals with Abnormal Proteins

  2007cites this paper
• Hepatocellular carcinoma pathogenesis: from genes to environment

  2006cites this paper
• Ubiquitin ligase gp78 increases solubility and facilitates degradation of the Z variant of alpha-1-antitrypsin.

  2006cites this paper
• Identification of proteins adducted by reactive metabolites of naphthalene and 1‐nitronaphthalene in dissected airways of rhesus macaques

  2006cites this paper
• Strategies for dissecting genetic-environmental interactions in neurodegenerative disorders.

  2006cites this paper
• Characterization of serine/cysteine protease inhibitor α1‐antitripsin from meconium‐instilled rabbit lungs

  2005cites this paper
• [Alpha 1-proteinase inhibitor deficiency and isolated liver cirrhosis without pulmonary emphysema in a 53-year-old female patient].

  2005cites this paper
• Alpha‐1 anti‐trypsin deficiency and Henoch‐Schönlein purpura associated with anti‐neutrophil cytoplasmic and anti‐endothelial cell antibodies of immunoglobulin‐A isotype

  2005cites this paper
• Characterization of recombinant human protein C inhibitor expressed in Escherichia coli.

  2005cites this paper
• Rapid separation of protein isoforms by capillary zone electrophoresis with new dynamic coatings

  2005cites this paper
• Biological markers for metal toxicity.

  2005cites this paper
• Alpha 1-Proteinaseninhibitormangel und isolierte Leberzirrhose ohne Lungenemphysem bei einer 53-jährigen Patientin

  2005cites this paper
• Trends in the diagnosis of symptomatic patients with alpha1-antitrypsin deficiency between 1968 and 2003.

  2005cites this paper
• Human EDEM2, a novel homolog of family 47 glycosidases, is involved in ER-associated degradation of glycoproteins.

  2005cites this paper
• Exceptional matters: clinical research from bedside to bench.

  2004cites this paper
• alpha1-Antitrypsin deficiency . 6: new and emerging treatments for alpha1-antitrypsin deficiency.

  2004cites this paper
• Exceptional matters

  2004cites this paper
• Dorsoventral patterning: a serpin pinned down at last.

  2004cites this paper
• Predicting subcellular localization via protein motif co-occurrence.

  2004cites this paper
• Receptor rigidity and ligand mobility in trypsin–ligand complexes

  2004cites this paper
• Regulation of Antiprotease and Antimicrobial Protein Secretion by Airway Submucosal Gland Serous Cells*

  2004cites this paper
• Divergent effects of alpha1-antitrypsin on neutrophil activation, in vitro.

  2004cites this paper
• Prevalence of alpha1-antitrypsin phenotypes in patients with IgA nephropathy.

  2004cites this paper
• Inhibition of lipopolysaccharide-mediated human monocyte activation, in vitro, by alpha1-antitrypsin.

  2004cites this paper
• Environmental, occupational, and genetic risk factors for alpha-1 antitrypsin deficiency.

  2003cites this paper
• Cirrhosis of the Liver

  1943cites this paper
• In vitro Sustained Release of alpha 1-antitrypsin from Poly ( D , L-lactide-co-glycolide ) Nanoparticles

  year unknowncites this paper