Alpha-1-antitrypsin deficiency: biochemistry and clinical manifestations (Q41250313)

28 September 2017

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title

Alpha-1-antitrypsin deficiency: biochemistry and clinical manifestations (English)

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alpha-1-antitrypsin deficiency

28 September 2017

main subject

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Perlmutter DH

1

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28 September 2017

language of work or name

English

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publication date

1 October 1996

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28 September 2017

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28 September 2017

volume

28

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28 September 2017

issue

5

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28 September 2017

page(s)

385-394

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Smoking, lung function, and alpha 1-antitrypsin deficiency.

28 September 2017

cites work

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Antielastases of the human alveolar structures. Implications for the protease-antiprotease theory of emphysema

21 January 2018

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The effect of amino acid substitutions at position 342 on the secretion of human alpha 1-antitrypsin from Xenopus oocytes

21 January 2018

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Accumulation of PiZ alpha 1-antitrypsin causes liver damage in transgenic mice

21 January 2018

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Implications of the three-dimensional structure of alpha 1-antitrypsin for structure and function of serpins

21 January 2018

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Liver transplantation for advanced liver disease with alpha-1-antitrypsin deficiency

21 January 2018

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Expression of the alpha 1-proteinase inhibitor gene in human monocytes and macrophages

21 January 2018

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Cell-specific expression of alpha 1-antitrypsin in human intestinal epithelium

21 January 2018

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Identification of a serpin-enzyme complex receptor on human hepatoma cells and human monocytes

21 January 2018

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The serpin-enzyme complex (SEC) receptor mediates the neutrophil chemotactic effect of alpha-1 antitrypsin-elastase complexes and amyloid-beta peptide

21 January 2018

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Specificity in recognition of amyloid-beta peptide by the serpin-enzyme complex receptor in hepatoma cells and neuronal cells

21 January 2018

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Differential recognition of alpha 1-antitrypsin-elastase and alpha 1-antichymotrypsin-cathepsin G complexes by the low density lipoprotein receptor-related protein

21 January 2018

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Molecular basis of alpha-1-antitrypsin deficiency

21 January 2018

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Mutation of antitrypsin to antithrombin. alpha 1-antitrypsin Pittsburgh (358 Met leads to Arg), a fatal bleeding disorder

21 January 2018

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The mechanism of Z alpha 1-antitrypsin accumulation in the liver

21 January 2018

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Conceptual advances in the pathogenesis and treatment of childhood metabolic liver disease.

21 January 2018

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alpha 1-Antitrypsin Mmalton (Phe52-deleted) forms loop-sheet polymers in vivo. Evidence for the C sheet mechanism of polymerization

21 January 2018

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Mutations which impede loop/sheet polymerization enhance the secretion of human alpha 1-antitrypsin deficiency variants

21 January 2018

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COOH-terminal substitutions in the serpin C1 inhibitor that cause loop overinsertion and subsequent multimerization

21 January 2018

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The rate of bulk flow from the endoplasmic reticulum to the cell surface

21 January 2018

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Polypeptide chain binding proteins: catalysts of protein folding and related processes in cells

21 January 2018

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Participation of a novel 88-kD protein in the biogenesis of murine class I histocompatibility molecules

21 January 2018

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Endoplasmic reticulum resident protein of 90 kilodaltons associates with the T- and B-cell antigen receptors and major histocompatibility complex antigens during their assembly

21 January 2018

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Association of folding intermediates of glycoproteins with calnexin during protein maturation

21 January 2018

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The molecular chaperone calnexin binds Glc1Man9GlcNAc2 oligosaccharide as an initial step in recognizing unfolded glycoproteins

21 January 2018

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Glucose trimming and reglucosylation determine glycoprotein association with calnexin in the endoplasmic reticulum

21 January 2018

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Pre-Golgi degradation of newly synthesized T-cell antigen receptor chains: intrinsic sensitivity and the role of subunit assembly

21 January 2018

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Misfolded major histocompatibility complex class I molecules accumulate in an expanded ER-Golgi intermediate compartment

21 January 2018

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Synthesis of stress proteins is increased in individuals with homozygous PiZZ alpha 1-antitrypsin deficiency and liver disease

21 January 2018

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A lag in intracellular degradation of mutant alpha 1-antitrypsin correlates with the liver disease phenotype in homozygous PiZZ alpha 1-antitrypsin deficiency

21 January 2018

1 reference

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Two pathways for the degradation of the H2 subunit of the asialoglycoprotein receptor in the endoplasmic reticulum

21 January 2018

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Retroviral-mediated transfer and expression of hepatitis B e antigen in human primary skin fibroblasts and Epstein-Barr virus-transformed B lymphocytes

21 January 2018

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Degradation of CFTR by the ubiquitin-proteasome pathway

21 January 2018

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Multiple proteolytic systems, including the proteasome, contribute to CFTR processing

21 January 2018

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Molecular pathogenesis of hepatocellular carcinoma in hepatitis B virus transgenic mice

21 January 2018

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Recombinant DNA-produced alpha 1-antitrypsin administered by aerosol augments lower respiratory tract antineutrophil elastase defenses in individuals with alpha 1-antitrypsin deficiency

21 January 2018

1 reference

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Replacement therapy for alpha 1-antitrypsin deficiency associated with emphysema

21 January 2018

1 reference

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Alpha 1-antitrypsin deficiency, emphysema, and liver disease. Genetic basis and strategies for therapy

21 January 2018

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Site-directed mutagenesis by gene targeting in mouse embryo-derived stem cells

21 January 2018

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Ribozyme-mediated repair of defective mRNA by targeted, trans-splicing

21 January 2018

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10.3109/07853899608999097

21 January 2018

Identifiers

DOI

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28 September 2017

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