(Q36444154)

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:26494620%20AND%20SRC:MED&resulttype=core&format=json

28 February 2020

title

Enhancing Autophagy with Drugs or Lung-directed Gene Therapy Reverses the Pathological Effects of Respiratory Epithelial Cell Proteinopathy (English)

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:26494620%20AND%20SRC:MED&resulttype=core&format=json

28 February 2020

0 references

0 references

1

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:26494620%20AND%20SRC:MED&resulttype=core&format=json

28 February 2020

Naftali Kaminski

14

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:26494620%20AND%20SRC:MED&resulttype=core&format=json

28 February 2020

author name string

Donna B Stolz

2

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:26494620%20AND%20SRC:MED&resulttype=core&format=json

28 February 2020

John F Alcorn

3

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:26494620%20AND%20SRC:MED&resulttype=core&format=json

28 February 2020

Samuel A Yousem

4

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:26494620%20AND%20SRC:MED&resulttype=core&format=json

28 February 2020

Jieru Wang

5

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:26494620%20AND%20SRC:MED&resulttype=core&format=json

28 February 2020

Adriana S Leme

6

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:26494620%20AND%20SRC:MED&resulttype=core&format=json

28 February 2020

A McGarry Houghton

7

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:26494620%20AND%20SRC:MED&resulttype=core&format=json

28 February 2020

Pamela Hale

8

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:26494620%20AND%20SRC:MED&resulttype=core&format=json

28 February 2020

Michael Ewing

9

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:26494620%20AND%20SRC:MED&resulttype=core&format=json

28 February 2020

Houming Cai

10

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:26494620%20AND%20SRC:MED&resulttype=core&format=json

28 February 2020

Evelyn Akpadock Garchar

11

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:26494620%20AND%20SRC:MED&resulttype=core&format=json

28 February 2020

Nunzia Pastore

12

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:26494620%20AND%20SRC:MED&resulttype=core&format=json

28 February 2020

Patrizia Annunziata

13

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:26494620%20AND%20SRC:MED&resulttype=core&format=json

28 February 2020

Joseph Pilewski

15

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:26494620%20AND%20SRC:MED&resulttype=core&format=json

28 February 2020

Steven D Shapiro

16

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:26494620%20AND%20SRC:MED&resulttype=core&format=json

28 February 2020

Stephen C Pak

17

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:26494620%20AND%20SRC:MED&resulttype=core&format=json

28 February 2020

Gary A Silverman

18

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:26494620%20AND%20SRC:MED&resulttype=core&format=json

28 February 2020

Nicola Brunetti-Pierri

19

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:26494620%20AND%20SRC:MED&resulttype=core&format=json

28 February 2020

David H Perlmutter

20

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:26494620%20AND%20SRC:MED&resulttype=core&format=json

28 February 2020

language of work or name

English

0 references

publication date

22 October 2015

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:26494620%20AND%20SRC:MED&resulttype=core&format=json

Journal of Biological Chemistry

28 February 2020

published in

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:26494620%20AND%20SRC:MED&resulttype=core&format=json

28 February 2020

volume

290

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:26494620%20AND%20SRC:MED&resulttype=core&format=json

28 February 2020

issue

50

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:26494620%20AND%20SRC:MED&resulttype=core&format=json

28 February 2020

page(s)

29742-29757

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:26494620%20AND%20SRC:MED&resulttype=core&format=json

describes a project that uses

28 February 2020

ImageJ

1 reference

11 June 2022

https://www.ebi.ac.uk/europepmc/webservices/rest/PMC4705969/fullTextXML

inferred from PubMed Central ID database lookup

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A C. elegans model of human α1-antitrypsin deficiency links components of the RNAi pathway to misfolded protein turnover

30 September 2017

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Fluphenazine reduces proteotoxicity in C. elegans and mammalian models of alpha-1-antitrypsin deficiency

30 September 2017

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30 September 2017

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IL-22 is essential for lung epithelial repair following influenza infection

30 September 2017

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Gene transfer of master autophagy regulator TFEB results in clearance of toxic protein and correction of hepatic disease in alpha-1-anti-trypsin deficiency

30 September 2017

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The alveolar epithelium determines susceptibility to lung fibrosis in Hermansky-Pudlak syndrome

30 September 2017

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Ligand-independent transforming growth factor-β type I receptor signalling mediates type I collagen-induced epithelial-mesenchymal transition

30 September 2017

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Why has it been so difficult to prove the efficacy of alpha-1-antitrypsin replacement therapy? Insights from the study of disease pathogenesis

30 September 2017

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Prostaglandin E₂ protects murine lungs from bleomycin-induced pulmonary fibrosis and lung dysfunction

30 September 2017

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Alveolar epithelial cells undergo epithelial-to-mesenchymal transition in response to endoplasmic reticulum stress

30 September 2017

1 reference

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Endoplasmic reticulum stress enhances fibrotic remodeling in the lungs

30 September 2017

1 reference

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TFEB links autophagy to lysosomal biogenesis

30 September 2017

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Lung volumes and emphysema in smokers with interstitial lung abnormalities

30 September 2017

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Role of endoplasmic reticulum stress in epithelial-mesenchymal transition of alveolar epithelial cells: effects of misfolded surfactant protein

30 September 2017

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Alpha-1-antitrypsin deficiency: importance of proteasomal and autophagic degradative pathways in disposal of liver disease-associated protein aggregates

30 September 2017

1 reference

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Impaired autophagy in sporadic inclusion-body myositis and in endoplasmic reticulum stress-provoked cultured human muscle fibers

30 September 2017

1 reference

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Intravenous alpha-1 antitrypsin augmentation therapy for treating patients with alpha-1 antitrypsin deficiency and lung disease

30 September 2017

1 reference

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An autophagy-enhancing drug promotes degradation of mutant alpha1-antitrypsin Z and reduces hepatic fibrosis.

30 September 2017

1 reference

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Large-scale production of high-quality helper-dependent adenoviral vectors using adherent cells in cell factories

30 September 2017

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Clinical practice. Alpha1-antitrypsin deficiency

30 September 2017

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Genetic defects in surfactant protein A2 are associated with pulmonary fibrosis and lung cancer.

30 September 2017

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How to interpret LC3 immunoblotting

30 September 2017

1 reference

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Determinants of airflow obstruction in severe alpha-1-antitrypsin deficiency

30 September 2017

1 reference

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A pure population of lung alveolar epithelial type II cells derived from human embryonic stem cells

30 September 2017

1 reference

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Differentiated human alveolar epithelial cells and reversibility of their phenotype in vitro

30 September 2017

1 reference

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Increased and prolonged pulmonary fibrosis in surfactant protein C-deficient mice following intratracheal bleomycin

30 September 2017

1 reference

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Accumulation of mutant alpha1-antitrypsin Z in the endoplasmic reticulum activates caspases-4 and -12, NFkappaB, and BAP31 but not the unfolded protein response.

30 September 2017

1 reference

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Impairment of starvation-induced and constitutive autophagy in Atg7-deficient mice

30 September 2017

1 reference

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Defective surfactant secretion in a mouse model of Hermansky-Pudlak syndrome

30 September 2017

1 reference

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Polymers of Z alpha1-antitrypsin co-localize with neutrophils in emphysematous alveoli and are chemotactic in vivo

30 September 2017

1 reference

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Carbon monoxide suppresses bleomycin-induced lung fibrosis

30 September 2017

1 reference

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The bronchopulmonary pathology of alpha-1 antitrypsin (AAT) deficiency: findings of the Death Review Committee of the national registry for individuals with Severe Deficiency of Alpha-1 Antitrypsin

30 September 2017

1 reference

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In vivo analysis of autophagy in response to nutrient starvation using transgenic mice expressing a fluorescent autophagosome marker

30 September 2017

1 reference

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Beclin 1, an autophagy gene essential for early embryonic development, is a haploinsufficient tumor suppressor

30 September 2017

1 reference

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Promotion of tumorigenesis by heterozygous disruption of the beclin 1 autophagy gene

30 September 2017

1 reference

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Neutrophil elastase contributes to cigarette smoke-induced emphysema in mice

30 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4705969

Improved system for helper-dependent adenoviral vector production

30 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4705969

Expression of a Human Surfactant Protein C Mutation Associated with Interstitial Lung Disease Disrupts Lung Development in Transgenic Mice

30 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4705969

Sputum chemotactic activity in chronic obstructive pulmonary disease: effect of alpha(1)-antitrypsin deficiency and the role of leukotriene B(4) and interleukin 8

30 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4705969

Cell-specific involvement of HNF-1beta in alpha(1)-antitrypsin gene expression in human respiratory epithelial cells

30 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4705969

Caspase-12 mediates endoplasmic-reticulum-specific apoptosis and cytotoxicity by amyloid-beta

30 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4705969

Cellular and connective tissue changes in alveolar septal walls in emphysema

30 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4705969

Biosynthesis of alpha1-proteinase inhibitor by human lung-derived epithelial cells

30 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4705969

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

30 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4705969

Multiple tissues express alpha 1-antitrypsin in transgenic mice and man.

30 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4705969

Neutrophil accumulation in the lung in alpha 1-antitrypsin deficiency. Spontaneous release of leukotriene B4 by alveolar macrophages

30 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4705969

Small airways in idiopathic pulmonary fibrosis. Comparison of morphologic and physiologic observations

30 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4705969

Induced pluripotent stem cells model personalized variations in liver disease resulting from α1-antitrypsin deficiency.

30 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4705969

Enhanced autophagy ameliorates cardiac proteinopathy.

28 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4705969

Results of a survey of patients with alpha-1 antitrypsin deficiency.

28 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4705969

Lung polymers in Z alpha1-antitrypsin deficiency-related emphysema

28 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4705969

Widespread expression of human alpha 1-antitrypsin in transgenic mice revealed by in situ hybridization.

28 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4705969

Outcomes for recipients of liver transplantation for alpha-1-antitrypsin deficiency–related cirrhosis.

28 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4705969

Autophagy-enhancing drug carbamazepine diminishes hepatocellular death in fibrinogen storage disease.

21 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4705969

The Registry of the International Society for Heart and Lung Transplantation: 29th adult lung and heart-lung transplant report-2012.

21 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4705969

Efficient and rapid isolation and purification of mouse alveolar type II epithelial cells.

21 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4705969

Basement membrane remodelling and segmental fibrosis in sporadic inclusion body myositis.

21 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4705969

Exploring the role of CT densitometry: a randomised study of augmentation therapy in alpha1-antitrypsin deficiency.

21 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4705969

Regulator of G Signaling 16 is a marker for the distinct endoplasmic reticulum stress state associated with aggregated mutant alpha1-antitrypsin Z in the classical form of alpha1-antitrypsin deficiency.

21 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4705969

COX-2-derived prostacyclin protects against bleomycin-induced pulmonary fibrosis

21 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4705969

Readministration of helper-dependent adenovirus to mouse lung

21 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4705969

Alpha1-antitrypsin deficiency in 26-year-old subjects: lung, liver, and protease/protease inhibitor studies

21 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4705969

Z alpha1-antitrypsin polymerizes in the lung and acts as a neutrophil chemoattractant

21 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4705969

Acute toxicity after high-dose systemic injection of helper-dependent adenoviral vectors into nonhuman primates.

21 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4705969

Pharmacological modulation of ion transport across wild-type and DeltaF508 CFTR-expressing human bronchial epithelia

21 September 2018

1 reference

12 December 2020

Longitudinal follow-up of patients with alpha(1)-protease inhibitor deficiency before and during therapy with IV alpha(1)-protease inhibitor

1 reference

12 December 2020

IKKβ Is Required for Prevention of Apoptosis Mediated by Cell-Bound but Not by Circulating TNFα

1 reference

12 December 2020

Intracellular inclusions containing mutant alpha1-antitrypsin Z are propagated in the absence of autophagic activity

1 reference

12 December 2020

Survival benefit of lung transplantation in individuals with severe α1-anti-trypsin deficiency (PiZZ) and emphysema

1 reference

12 December 2020

Smoking and subclinical interstitial lung disease

1 reference

12 December 2020

Identifiers

DOI

10.1074/JBC.M115.691253

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:26494620%20AND%20SRC:MED&resulttype=core&format=json

28 February 2020

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:26494620%20AND%20SRC:MED&resulttype=core&format=json

28 February 2020

1 reference