(Q37056312)

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:18193338%20AND%20SRC:MED&resulttype=core&format=json

31 December 2019

review article

1 reference

title

Alpha-1 antitrypsin deficiency: a conformational disease associated with lung and liver manifestations (English)

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:18193338%20AND%20SRC:MED&resulttype=core&format=json

alpha-1-antitrypsin deficiency

31 December 2019

main subject

1 reference

1

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:18193338%20AND%20SRC:MED&resulttype=core&format=json

31 December 2019

Tomás P. Carroll

3

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:18193338%20AND%20SRC:MED&resulttype=core&format=json

31 December 2019

Clifford C Taggart

8

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:18193338%20AND%20SRC:MED&resulttype=core&format=json

31 December 2019

author name string

S D W Miller

2

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:18193338%20AND%20SRC:MED&resulttype=core&format=json

31 December 2019

C McLean

4

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:18193338%20AND%20SRC:MED&resulttype=core&format=json

31 December 2019

M O'Mahony

5

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:18193338%20AND%20SRC:MED&resulttype=core&format=json

31 December 2019

M W Lawless

6

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:18193338%20AND%20SRC:MED&resulttype=core&format=json

31 December 2019

S J O'Neill

7

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:18193338%20AND%20SRC:MED&resulttype=core&format=json

31 December 2019

N G McElvaney

9

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:18193338%20AND%20SRC:MED&resulttype=core&format=json

31 December 2019

publication date

16 January 2008

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:18193338%20AND%20SRC:MED&resulttype=core&format=json

Journal of Inherited Metabolic Disease

31 December 2019

published in

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:18193338%20AND%20SRC:MED&resulttype=core&format=json

31 December 2019

volume

31

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:18193338%20AND%20SRC:MED&resulttype=core&format=json

31 December 2019

issue

1

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:18193338%20AND%20SRC:MED&resulttype=core&format=json

31 December 2019

page(s)

21-34

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:18193338%20AND%20SRC:MED&resulttype=core&format=json

https://scigraph.springernature.com/pub.10.1007/s10545-007-0748-y

31 December 2019

exact match

0 references

cites work

Clinical features and history of the destructive lung disease associated with alpha-1-antitrypsin deficiency of adults with pulmonary symptoms.

1 reference

https://api.crossref.org/works/10.1007%2FS10545-007-0748-Y

Lung polymers in Z alpha1-antitrypsin deficiency-related emphysema

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS10545-007-0748-Y

Activation of endoplasmic reticulum-specific stress responses associated with the conformational disease Z alpha 1-antitrypsin deficiency.

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS10545-007-0748-Y

A pilot study of all-trans-retinoic acid for the treatment of human emphysema

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS10545-007-0748-Y

Maximal static respiratory pressures in the normal elderly.

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS10545-007-0748-Y

Global strategy for the diagnosis, management, and prevention of chronic obstructive pulmonary disease. NHLBI/WHO Global Initiative for Chronic Obstructive Lung Disease (GOLD) Workshop summary

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS10545-007-0748-Y

High prevalence of viral infection in adults with homozygous and heterozygous alpha 1-antitrypsin deficiency and chronic liver disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS10545-007-0748-Y

Translational control in the endoplasmic reticulum stress response

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS10545-007-0748-Y

ER stress and the unfolded protein response

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS10545-007-0748-Y

Variability of pulmonary function in alpha-1-antitrypsin deficiency: clinical correlates.

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS10545-007-0748-Y

Fasting in alpha1-antitrypsin deficient liver: constitutive [correction of consultative] activation of autophagy.

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS10545-007-0748-Y

High resolution computed tomography (HRCT) in emphysema associated with alpha-1-antitrypsin deficiency

21 January 2018

1 reference

12 December 2020

The proteasome participates in degradation of mutant alpha 1-antitrypsin Z in the endoplasmic reticulum of hepatoma-derived hepatocytes

1 reference

12 December 2020

Antineutrophil cytoplasmic antibodies induce monocyte IL-8 release. Role of surface proteinase-3, alpha1-antitrypsin, and Fcgamma receptors

1 reference

12 December 2020

Pathogenesis and pathology of liver disease associated with alpha 1-antitrypsin deficiency

1 reference

12 December 2020

Serum alpha 1-antitrypsin deficiency associated with the common S-type (Glu264----Val) mutation results from intracellular degradation of alpha 1-antitrypsin prior to secretion

1 reference

12 December 2020

EDEM as an acceptor of terminally misfolded glycoproteins released from calnexin

1 reference

12 December 2020

Vitamin E deficiency in adults with chronic liver disease

1 reference

12 December 2020

Anti-neutrophil-elastase defenses of the lower respiratory tract in alpha 1-antitrypsin deficiency directly augmented with an aerosol of alpha 1-antitrypsin

1 reference

12 December 2020

In vivo post-transcriptional gene silencing of alpha-1 antitrypsin by adeno-associated virus vectors expressing siRNA.

1 reference

12 December 2020

Tauroursodeoxycholic acid inhibits apoptosis induced by Z alpha-1 antitrypsin via inhibition of Bad.

1 reference

12 December 2020

Alpha 1-antitrypsin deficiency: the radiological features of pulmonary emphysema in subjects of Pi type Z and Pi type SZ: a survey by the British Thoracic Association

1 reference

12 December 2020

The liver in adolescents with alpha 1-antitrypsin deficiency

1 reference

12 December 2020

Enhanced phenotypic expression of alpha-1-antitrypsin deficiency in an MZ heterozygote with chronic hepatitis C.

1 reference

12 December 2020

alpha 1-Antitrypsin: molecular pathology, leukocytes, and tissue damage

1 reference

12 December 2020

Baseline characteristics of enrollees in the National Heart, Lung and Blood Institute Registry of alpha 1-antitrypsin deficiency. Alpha 1-Antitrypsin Deficiency Registry Study Group

1 reference

12 December 2020

Targeting a surface cavity of alpha 1-antitrypsin to prevent conformational disease

1 reference

12 December 2020

Alpha 1 antitrypsin deficiency: the clinical and physiological features of pulmonary emphysema in subjects homozygous for Pi type Z. A survey by the British Thoracic Association

1 reference

12 December 2020

The liver in noncirrhotic -1-antitrypsin deficiency

1 reference

12 December 2020

Mutants of neuroserpin that cause dementia accumulate as polymers within the endoplasmic reticulum

1 reference

12 December 2020

Prenatal diagnosis of alpha 1 antitrypsin deficiency and estimates of fetal risk for disease

1 reference

12 December 2020

Commercialization of proteins produced in the mammary gland

1 reference

12 December 2020

Variable degree of liver involvement in siblings with PiZZ alpha-1-antitrypsin deficiency-related liver disease

1 reference

12 December 2020

Retention of mutant alpha(1)-antitrypsin Z in endoplasmic reticulum is associated with an autophagic response

1 reference

12 December 2020

Sustained secretion of human alpha-1-antitrypsin from murine muscle transduced with adeno-associated virus vectors

1 reference

12 December 2020

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

1 reference

12 December 2020

Serine protease inhibitors in patients with chronic viral hepatitis

1 reference

12 December 2020

Synthetic inhibitors of elastase

1 reference

12 December 2020

6-mer peptide selectively anneals to a pathogenic serpin conformation and blocks polymerization. Implications for the prevention of Z alpha(1)-antitrypsin-related cirrhosis

1 reference

12 December 2020

Late manifestation of chronic liver disease in adults with alpha-1-antitrypsin deficiency

1 reference

12 December 2020

Chromosomal localization of the human alpha 1-antitrypsin gene (PI) to 14q31-32.

1 reference

12 December 2020

In-frame single codon deletion in the Mmalton deficiency allele of alpha 1-antitrypsin

1 reference

12 December 2020

Activating transcription factor 3 is integral to the eukaryotic initiation factor 2 kinase stress response

1 reference

12 December 2020

Cirrhosis associated with alpha-1-antitrypsin deficiency: a previously unrecognized inherited disorder

1 reference

12 December 2020

Heterozygous MZ alpha-1-antitrypsin deficiency in adults with chronic liver disease

1 reference

12 December 2020

ONO-5046, a novel inhibitor of human neutrophil elastase

1 reference

12 December 2020

Inhibition of intracellular degradation increases secretion of a mutant form of alpha1-antitrypsin associated with profound deficiency

1 reference

12 December 2020

Modulation of airspace enlargement in elastase-induced emphysema by intratracheal instillment of hyaluronidase and hyaluronic acid

1 reference

12 December 2020

The unfolded protein response is involved in the pathology of Alzheimer's disease.

1 reference

12 December 2020

Mitochondrial autophagy and injury in the liver in alpha 1-antitrypsin deficiency.

1 reference

12 December 2020

Ribozyme-mediated specific gene replacement of the alpha1-antitrypsin gene in human hepatoma cells

1 reference

12 December 2020

XBP1 mRNA is induced by ATF6 and spliced by IRE1 in response to ER stress to produce a highly active transcription factor

1 reference

12 December 2020

PULMONARY EMPHYSEMA AND ALPHA1-ANTITRYPSIN DEFICIENCY.

1 reference

12 December 2020

Prevention of polymerization of M and Z alpha1-Antitrypsin (alpha1-AT) with trimethylamine N-oxide. Implications for the treatment of alpha1-at deficiency

1 reference

12 December 2020

Alpha-1-antitrypsin deficiency: a new paradigm for hepatocellular carcinoma in genetic liver disease

1 reference

12 December 2020

Liver Disease in Alpha1-Antitrypsin Deficiency Detected by Screening of 200,000 Infants

1 reference

12 December 2020

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

1 reference

12 December 2020

Aerosolized hyaluronic acid decreases alveolar injury induced by human neutrophil elastase

1 reference

12 December 2020

MR889, a neutrophil elastase inhibitor, in patients with chronic obstructive pulmonary disease: a double-blind, randomized, placebo-controlled clinical trial

1 reference

12 December 2020

Long-term follow-up of a cohort of children with alpha-1-antitrypsin deficiency

1 reference

12 December 2020

The relationship of chronic sputum expectoration to physiologic, radiologic, and health status characteristics in alpha(1)-antitrypsin deficiency (PiZ).

1 reference

12 December 2020

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

1 reference

12 December 2020

Two distinct stress signaling pathways converge upon the CHOP promoter during the mammalian unfolded protein response

1 reference

12 December 2020

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

1 reference

12 December 2020

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

1 reference

12 December 2020

The isolation of a clone for human alpha 1-antitrypsin and the detection of alpha 1-antitrypsin in mRNA from liver and leukocytes

1 reference

12 December 2020

Tripartite management of unfolded proteins in the endoplasmic reticulum

1 reference

12 December 2020

Does alpha1-antitrypsin phenotype PiMZ increase the risk of fibrosis in liver disease due to hepatitis C virus infection?

1 reference

12 December 2020

The electrophoretic α1-globulin pattern of serum in α1-antitrypsin deficiency. 1963.

1 reference

12 December 2020

Respiratory symptoms and lung function in young adults with severe alpha(1)-antitrypsin deficiency (PiZZ)

1 reference

12 December 2020

Alpha 1-antitrypsin deficiency in Europe: geographical distribution of Pi types S and Z

1 reference

12 December 2020

Alpha1-antitrypsin deficiency--a model for conformational diseases

1 reference

12 December 2020

Strategies for aerosol therapy of alpha 1-antitrypsin deficiency by the aerosol route

1 reference

12 December 2020

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

1 reference

12 December 2020

DeltaF508-CFTR causes constitutive NF-kappaB activation through an ER-overload response in cystic fibrosis lungs

1 reference

12 December 2020

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

1 reference

12 December 2020

Sugar and alcohol molecules provide a therapeutic strategy for the serpinopathies that cause dementia and cirrhosis

1 reference

12 December 2020

Heterozygous alpha 1-antitrypsin phenotypes in patients with end stage liver disease.

1 reference

12 December 2020

Biologic characterization of ICI 200,880 and ICI 200,355, novel inhibitors of human neutrophil elastase

1 reference

12 December 2020

ER quality control: the cytoplasmic connection

1 reference

12 December 2020

Outcome of liver disease associated with alpha 1 antitrypsin deficiency (PiZ). Implications for genetic counselling and antenatal diagnosis

1 reference

12 December 2020

A time-dependent phase shift in the mammalian unfolded protein response

1 reference

12 December 2020

Lung neutrophil burden correlates with increased pro-inflammatory cytokines and decreased lung function in individuals with alpha(1)-antitrypsin deficiency

1 reference

12 December 2020

Enhancement of endoplasmic reticulum (ER) degradation of misfolded Null Hong Kong alpha1-antitrypsin by human ER mannosidase I.

1 reference

12 December 2020

Role of ubiquitin in proteasomal degradation of mutant alpha(1)-antitrypsin Z in the endoplasmic reticulum

1 reference

12 December 2020

Frequency and significance of phenotypes for alpha1-antitrypsin deficiency in type 1 autoimmune hepatitis

1 reference

12 December 2020

Worldwide racial and ethnic distribution of alpha1-antitrypsin deficiency: summary of an analysis of published genetic epidemiologic surveys

1 reference

12 December 2020

Detection of circulating and endothelial cell polymers of Z and wild type alpha 1-antitrypsin by a monoclonal antibody

1 reference

12 December 2020

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

1 reference

12 December 2020

Liver injury in alpha1-antitrypsin deficiency: an aggregated protein induces mitochondrial injury

1 reference

12 December 2020

Grp78, Grp94, and Grp170 interact with alpha1-antitrypsin mutants that are retained in the endoplasmic reticulum

1 reference

12 December 2020

Augmentation of lung antineutrophil elastase capacity with recombinant human alpha-1-antitrypsin

1 reference

12 December 2020

Functional and genomic analyses reveal an essential coordination between the unfolded protein response and ER-associated degradation

1 reference

12 December 2020

Glucosidase and mannosidase inhibitors mediate increased secretion of mutant alpha1 antitrypsin Z.

1 reference

12 December 2020

Recombinant adeno-associated virus gene therapy for cystic fibrosis and alpha(1)-antitrypsin deficiency

1 reference

12 December 2020

A review of alpha-1 antitrypsin deficiency

1 reference

12 December 2020

Assignment of human alpha 1-antitrypsin to chromosome 14 by somatic cell hybrid analysis

1 reference

12 December 2020

Heterozygous alpha-I antitrypsin deficiency as a co-factor in the development of chronic liver disease: a review.

1 reference

12 December 2020

High level expression of active human alpha-1-antitrypsin in the milk of transgenic sheep

1 reference

12 December 2020

Inhibition of Neutrophil Serine Proteinases by Suramin

1 reference

12 December 2020

Perk is essential for translational regulation and cell survival during the unfolded protein response

1 reference

12 December 2020

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

1 reference

12 December 2020

Expression of hereditary hemochromatosis C282Y HFE protein in HEK293 cells activates specific endoplasmic reticulum stress responses

1 reference

12 December 2020

Replacement therapy for alpha 1-antitrypsin deficiency associated with emphysema

1 reference

12 December 2020

Rare alpha-1-antitrypsin phenotypes and liver-test abnormalities during infancy

1 reference

12 December 2020

Factors associated with advanced liver disease in adults with alpha1-antitrypsin deficiency

1 reference

12 December 2020

Transcriptional and translational control in the Mammalian unfolded protein response

1 reference

12 December 2020

A patient with autoimmune hepatitis type I, Addison's disease, atrophic thyroiditis, atrophic gastritis, exocrine pancreatic insufficiency, and heterozygous alpha1-antitrypsin deficiency

1 reference

12 December 2020

Inhibition of lipopolysaccharide-mediated human monocyte activation, in vitro, by alpha1-antitrypsin

1 reference

12 December 2020