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Literature DB >> 26846838

Polymers of Z α1-antitrypsin are secreted in cell models of disease.

Annamaria Fra¹, Francesca Cosmi², Adriana Ordoñez³, Romina Berardelli¹, Juan Perez⁴, Noemi A Guadagno⁵, Luciano Corda⁶, Stefan J Marciniak⁷, David A Lomas⁸, Elena Miranda⁹.

Abstract

Entities: Gene

Mesh：

Substances：

Year: 2016 PMID： 26846838 DOI： 10.1183/13993003.00940-2015

Source DB: PubMed Journal: Eur Respir J ISSN： 0903-1936 Impact factor: 16.671

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17 in total

1. Modulation of calreticulin expression reveals a novel exosome-mediated mechanism of Z variant α₁-antitrypsin disposal.

Authors: Nazli Khodayari; Regina Oshins; Abdel A Alli; Kubra M Tuna; L Shannon Holliday; Karina Krotova; Mark Brantly
Journal: J Biol Chem Date: 2019-03-04 Impact factor: 5.157

2. Secretion of functional α1-antitrypsin is cell type dependent: Implications for intramuscular delivery for gene therapy.

Authors: Haiping Ke; Kevin P Guay; Terence R Flotte; Lila M Gierasch; Anne Gershenson; Daniel N Hebert
Journal: Proc Natl Acad Sci U S A Date: 2022-07-28 Impact factor: 12.779

3. Cargo receptor-assisted endoplasmic reticulum export of pathogenic α1-antitrypsin polymers.

Authors: Adriana Ordóñez; Heather P Harding; Stefan J Marciniak; David Ron
Journal: Cell Rep Date: 2021-05-18 Impact factor: 9.423

4. The pathological Trento variant of alpha-1-antitrypsin (E75V) shows nonclassical behaviour during polymerization.

Authors: Elena Miranda; Ilaria Ferrarotti; Romina Berardelli; Mattia Laffranchi; Marta Cerea; Fabrizio Gangemi; Imran Haq; Stefania Ottaviani; David A Lomas; James A Irving; Annamaria Fra
Journal: FEBS J Date: 2017-06-08 Impact factor: 5.542

Review 5. Update on α₁-antitrypsin deficiency.

Authors: Ilaria Ferrarotti; Stefania Ottaviani; Annalisa De Silvestri; Angelo G Corsico
Journal: Breathe (Sheff) Date: 2018-06

6. Characterisation of a type II functionally-deficient variant of alpha-1-antitrypsin discovered in the general population.

Authors: Mattia Laffranchi; Emma L K Elliston; Fabrizio Gangemi; Romina Berardelli; David A Lomas; James A Irving; Annamaria Fra
Journal: PLoS One Date: 2019-01-11 Impact factor: 3.752

Polymers of Z α1-antitrypsin are secreted in cell models of disease.

1. Modulation of calreticulin expression reveals a novel exosome-mediated mechanism of Z variant α₁-antitrypsin disposal.

2. Secretion of functional α1-antitrypsin is cell type dependent: Implications for intramuscular delivery for gene therapy.

3. Cargo receptor-assisted endoplasmic reticulum export of pathogenic α1-antitrypsin polymers.

4. The pathological Trento variant of alpha-1-antitrypsin (E75V) shows nonclassical behaviour during polymerization.

Review 5. Update on α₁-antitrypsin deficiency.

6. Characterisation of a type II functionally-deficient variant of alpha-1-antitrypsin discovered in the general population.

7. The Importance of N186 in the Alpha-1-Antitrypsin Shutter Region Is Revealed by the Novel Bologna Deficiency Variant.

Review 8. The Recruitment-Secretory Block ("R-SB") Phenomenon and Endoplasmic Reticulum Storage Diseases.

9. Intermittent C1-Inhibitor Deficiency Associated with Recessive Inheritance: Functional and Structural Insight.

10. Calcium signalling in mammalian cell lines expressing wild type and mutant human α1-Antitrypsin.

Review 5. Update on α1-antitrypsin deficiency.

Review 8. The Recruitment-Secretory Block ("R-SB") Phenomenon and Endoplasmic Reticulum Storage Diseases.

Review 5. Update on α₁-antitrypsin deficiency.