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

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Perlmutter DH

1

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PubMed ID

8949968

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

28

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28 September 2017

5

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28 September 2017

385-394

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

28 September 2017

1 reference

https://api.crossref.org/works/10.3109%2F07853899608999097

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

21 January 2018

1 reference

https://api.crossref.org/works/10.3109%2F07853899608999097

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

21 January 2018

1 reference

https://api.crossref.org/works/10.3109%2F07853899608999097

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

21 January 2018

1 reference

https://api.crossref.org/works/10.3109%2F07853899608999097

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

21 January 2018

1 reference

https://api.crossref.org/works/10.3109%2F07853899608999097

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

21 January 2018

1 reference

https://api.crossref.org/works/10.3109%2F07853899608999097

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

21 January 2018

1 reference

https://api.crossref.org/works/10.3109%2F07853899608999097

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

21 January 2018

1 reference

https://api.crossref.org/works/10.3109%2F07853899608999097

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

21 January 2018

1 reference

https://api.crossref.org/works/10.3109%2F07853899608999097

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

21 January 2018

1 reference

https://api.crossref.org/works/10.3109%2F07853899608999097

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

21 January 2018

1 reference

https://api.crossref.org/works/10.3109%2F07853899608999097

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

1 reference

https://api.crossref.org/works/10.3109%2F07853899608999097

Molecular basis of alpha-1-antitrypsin deficiency

21 January 2018

1 reference

https://api.crossref.org/works/10.3109%2F07853899608999097

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

21 January 2018

1 reference

https://api.crossref.org/works/10.3109%2F07853899608999097

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

21 January 2018

1 reference

https://api.crossref.org/works/10.3109%2F07853899608999097

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

21 January 2018

1 reference

https://api.crossref.org/works/10.3109%2F07853899608999097

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

21 January 2018

1 reference

https://api.crossref.org/works/10.3109%2F07853899608999097

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

21 January 2018

1 reference

https://api.crossref.org/works/10.3109%2F07853899608999097

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

21 January 2018

1 reference

https://api.crossref.org/works/10.3109%2F07853899608999097

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

21 January 2018

1 reference

https://api.crossref.org/works/10.3109%2F07853899608999097

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

21 January 2018

1 reference

https://api.crossref.org/works/10.3109%2F07853899608999097

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

21 January 2018

1 reference

https://api.crossref.org/works/10.3109%2F07853899608999097

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

1 reference

https://api.crossref.org/works/10.3109%2F07853899608999097

Association of folding intermediates of glycoproteins with calnexin during protein maturation

21 January 2018

1 reference

https://api.crossref.org/works/10.3109%2F07853899608999097

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

21 January 2018

1 reference

https://api.crossref.org/works/10.3109%2F07853899608999097

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

21 January 2018

1 reference

https://api.crossref.org/works/10.3109%2F07853899608999097

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

21 January 2018

1 reference

https://api.crossref.org/works/10.3109%2F07853899608999097

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

21 January 2018

1 reference

https://api.crossref.org/works/10.3109%2F07853899608999097

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

21 January 2018

1 reference

https://api.crossref.org/works/10.3109%2F07853899608999097

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

https://api.crossref.org/works/10.3109%2F07853899608999097

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

21 January 2018

1 reference

https://api.crossref.org/works/10.3109%2F07853899608999097

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

1 reference

https://api.crossref.org/works/10.3109%2F07853899608999097

Degradation of CFTR by the ubiquitin-proteasome pathway

21 January 2018

1 reference

https://api.crossref.org/works/10.3109%2F07853899608999097

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

21 January 2018

1 reference

https://api.crossref.org/works/10.3109%2F07853899608999097

Molecular pathogenesis of hepatocellular carcinoma in hepatitis B virus transgenic mice

21 January 2018

1 reference

https://api.crossref.org/works/10.3109%2F07853899608999097

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

https://api.crossref.org/works/10.3109%2F07853899608999097

Replacement therapy for alpha 1-antitrypsin deficiency associated with emphysema

21 January 2018

1 reference

https://api.crossref.org/works/10.3109%2F07853899608999097

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

21 January 2018

1 reference

https://api.crossref.org/works/10.3109%2F07853899608999097

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

21 January 2018

1 reference

https://api.crossref.org/works/10.3109%2F07853899608999097

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

21 January 2018

1 reference

https://api.crossref.org/works/10.3109%2F07853899608999097

10.3109/07853899608999097

21 January 2018

Identifiers

DOI

1 reference

28 September 2017

1 reference

PubMed ID

8949968