(Q36846798)

16 August 2017

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title

Alpha-1-antitrypsin deficiency: current concepts (English)

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alpha-1-antitrypsin deficiency

16 August 2017

main subject

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Clifford C Taggart

2

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author name string

Alan T Mulgrew

series ordinal

1

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16 August 2017

N Gerry McElvaney

series ordinal

3

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16 August 2017

publication date

12 June 2007

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16 August 2017

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16 August 2017

volume

185

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16 August 2017

issue

4

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16 August 2017

page(s)

191-201

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Lung polymers in Z alpha1-antitrypsin deficiency-related emphysema

16 August 2017

cites work

1 reference

https://api.crossref.org/works/10.1007%2FS00408-007-9009-Y

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS00408-007-9009-Y

Alpha-1-antitrypsin deficiency. High prevalence in the St. Louis area determined by direct population screening

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS00408-007-9009-Y

Bronchiectasis in patients with alpha 1-antitrypsin deficiency. A rare occurrence?

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS00408-007-9009-Y

Alpha 1-antitrypsin deficiency: evaluation of bronchiectasis with CT.

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS00408-007-9009-Y

Anti-proteinase 3 antibody activation of neutrophils can be inhibited by alpha1-antitrypsin.

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS00408-007-9009-Y

Oncostatin M is a potent stimulator of alpha1-antitrypsin secretion in lung epithelial cells: modulation by transforming growth factor-beta and interferon-gamma

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS00408-007-9009-Y

Polymers of alpha(1)-antitrypsin are chemotactic for human neutrophils: a new paradigm for the pathogenesis of emphysema

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS00408-007-9009-Y

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS00408-007-9009-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%2FS00408-007-9009-Y

Aerosol and intravenous transfection of human alpha 1-antitrypsin gene to lungs of rabbits

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS00408-007-9009-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

α 1 ‐antitrypsin deficiency and liver disease: Clinical presentation, diagnosis and treatment

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

Risk of cirrhosis and primary liver cancer in alpha 1-antitrypsin deficiency

1 reference

12 December 2020

Young adults with alpha 1-antitrypsin deficiency identified neonatally: their health, knowledge about and adaptation to the high-risk condition

1 reference

12 December 2020

Gene regulation of the serine proteinase inhibitors alpha1-antitrypsin and alpha1-antichymotrypsin

1 reference

12 December 2020

The protease-antiprotease balance within the human lung: implications for the pathogenesis of emphysema

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

Portacaval shunt in three children with alpha-1-antitrypsin deficiency and cirrhosis: 9 to 12 1/3 years later

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

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12 December 2020

The liver in adolescents with α-antitrypsin deficiency

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12 December 2020

Survival and FEV1 decline in individuals with severe deficiency of alpha1-antitrypsin. The Alpha-1-Antitrypsin Deficiency Registry Study Group

1 reference

12 December 2020

A canine model for production of severe unilateral panacinar emphysema

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

Progress of emphysema in severe alpha 1-antitrypsin deficiency as assessed by annual CT.

1 reference

12 December 2020

Pharmacological profile of the substituted beta-lactam L-659,286: a member of a new class of human PMN elastase inhibitors

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12 December 2020

α 1 ‐antitrypsin deficiency and liver disease

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12 December 2020

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

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12 December 2020

Alpha 1-antitrypsin: a guardian of vascular tissue

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12 December 2020

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

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12 December 2020

Hepatocyte Ultrastructural Changes in α1-Antitrypsin Deficiency

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12 December 2020

Clinical, clinical-chemical and pathological findings in familial alpha1-antitrypsin deficiency

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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

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12 December 2020

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

1 reference

12 December 2020

Phase I trial of intramuscular injection of a recombinant adeno-associated virus alpha 1-antitrypsin (rAAV2-CB-hAAT) gene vector to AAT-deficient adults

1 reference

12 December 2020

Variants of alpha 1-antitrypsin in Puerto Rican children with asthma

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12 December 2020

Cell migration, chimerism, and graft acceptance

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12 December 2020

The effect of augmentation therapy on bronchial inflammation in alpha1-antitrypsin deficiency

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12 December 2020

Efficient gene transfer into nondividing cells by adeno-associated virus-based vectors.

1 reference

12 December 2020

A regulatory link between ER-associated protein degradation and the unfolded-protein response

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12 December 2020

1 -Antitrypsin deficiency: a variant with no detectable 1 -antitrypsin

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12 December 2020

Alpha 1-antitrypsin and protease complexation is induced by lipopolysaccharide, interleukin-1beta, and tumor necrosis factor-alpha in monocytes

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12 December 2020

Hepatic gene therapy: efficient retroviral-mediated gene transfer into rat hepatocytes in vivo

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12 December 2020

Leukocyte kinetics in the human lung: role of exercise and catecholamines

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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

The effect of a peptide aldehyde reversible inhibitor of elastase on a human leucocyte elastase-induced model of emphysema in the hamster

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12 December 2020

α-1-antitrypsin phenotypes in hepatocellular carcinoma

1 reference

12 December 2020

Natural History and Life Expectancy in Severe Alpha1-Antitrypsin Deficiency, Pi Z

1 reference

12 December 2020

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

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12 December 2020

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

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12 December 2020

Structural explanation for the deficiency of S alpha 1-antitrypsin

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12 December 2020

Lung function, smoking and survival in severe alpha 1-antitrypsin deficiency, PiZZ

1 reference

12 December 2020

Liver, Lung and Malignant Disease in Heterozygous (Pi MZ) α1-antitrypsin Deficiency

1 reference

12 December 2020

Inhibiting polymerization: new therapeutic strategies for Z alpha1-antitrypsin-related emphysema

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12 December 2020

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

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12 December 2020

3-Hydroxy-3-methylglutaryl-coenzyme A reductase and T cell receptor alpha subunit are differentially degraded in the endoplasmic reticulum

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12 December 2020

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

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12 December 2020

Long-term results after lung volume reduction surgery in patients with alpha1-antitrypsin deficiency

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12 December 2020

Elastase in tissue injury

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12 December 2020

Emphysema induced in vitro and in vivo in dogs by a purified elastase from homologous leukocytes

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12 December 2020

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

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12 December 2020

Degradation of a mutant secretory protein, alpha1-antitrypsin Z, in the endoplasmic reticulum requires proteasome activity

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12 December 2020

Pathogenesis of chronic liver injury and hepatocellular carcinoma in alpha-1-antitrypsin deficiency

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

Short-term reproducibility of computed tomography-based lung density measurements in alpha-1 antitrypsin deficiency and smokers with emphysema

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12 December 2020

alpha1-Antitrypsin deficiency . 6: new and emerging treatments for alpha1-antitrypsin deficiency

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12 December 2020

Replacement therapy of alpha 1-antitrypsin deficiency. Reversal of protease-antiprotease imbalance within the alveolar structures of PiZ subjects

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12 December 2020

The serpin superfamily of proteinase inhibitors: structure, function, and regulation

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12 December 2020

Clonal gene therapy: transplanted mouse fibroblast clones express human alpha 1-antitrypsin gene in vivo

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12 December 2020

Strong link between the alpha 1-antitrypsin PiZ allele and Wegener's granulomatosis

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12 December 2020

The natural history of liver disease in alpha 1-antitrypsin deficient children

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12 December 2020

Alpha1-antitrypsin deficiency--a model for conformational diseases

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12 December 2020

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

1 reference

12 December 2020

Evidence-based medicine: lung volume reduction surgery (LVRS).

1 reference

12 December 2020

The inhibitory complex of human alpha 1-proteinase inhibitor and human leukocyte elastase is a neutrophil chemoattractant

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12 December 2020

Epidemiology of alpha 1-protease inhibitor deficiency

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12 December 2020

Inhibition of human leukocyte elastase (HLE) by N-substituted peptidyl trifluoromethyl ketones

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12 December 2020

Adenovirus-mediated transfer of a recombinant alpha 1-antitrypsin gene to the lung epithelium in vivo

1 reference

12 December 2020

Familial conformational diseases and dementias

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12 December 2020

Alpha1-antitrypsin and its deficiency

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12 December 2020

Absence of alpha-1-antitrypsin (Pi Null Bellingham) and the early onset of emphysema

1 reference

12 December 2020

Alpha-1-antitrypsin deficiency and panniculitis. Perspectives on disease relationship and replacement therapy

1 reference

12 December 2020

A future for neonatal alpha1-antitrypsin screening?

1 reference

12 December 2020

Cost-effectiveness analysis of augmentation therapy for severe alpha1-antitrypsin deficiency.

1 reference

12 December 2020

Risk for liver disease in adults with alpha 1-antitrypsin deficiency

1 reference

12 December 2020

Unilateral lung transplantation in end-stage pulmonary emphysema

1 reference

12 December 2020

Membranoproliferative glomerulonephritis and alpha 1-antitrypsin deficiency in children

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

Decline in pulmonary function in patients with alpha 1-antitrypsin deficiency.

1 reference

12 December 2020

Liver disease in alpha-1-antitrypsin deficiency: prognostic indicators

1 reference

12 December 2020

Morbidity, mortality, and early results of single versus bilateral lung transplantation for emphysema

1 reference

12 December 2020

Reversal of hepatic alpha-1-antitrypsin deposition after portacaval shunt

1 reference

12 December 2020

Rationale and design of the national emphysema treatment trial (NETT): A prospective randomized trial of lung volume reduction surgery

1 reference

12 December 2020

Adeno-associated virus: integration at a specific chromosomal locus

1 reference

12 December 2020

Alpha 1-antitrypsin deficiency and emphysema caused by homozygous inheritance of non-expressing alpha 1-antitrypsin genes

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

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

Thirteen-year experience in lung transplantation for emphysema.

1 reference

12 December 2020

Processing by endoplasmic reticulum mannosidases partitions a secretion-impaired glycoprotein into distinct disposal pathways

1 reference

12 December 2020

The natural history of air-flow obstruction in PiZ emphysema. Report of an NHLBI workshop

1 reference

12 December 2020

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

1 reference

12 December 2020

A novel signal transduction pathway from the endoplasmic reticulum to the nucleus is mediated by transcription factor NF-kappa B.

1 reference

12 December 2020

Acute phase protein stimulation by alpha 1-antichymotrypsin-cathepsin G complexes. Evidence for the involvement of interleukin-6.

1 reference

12 December 2020

Replacement therapy for alpha 1-antitrypsin deficiency associated with emphysema

1 reference

12 December 2020

The proteinase-antiproteinase balance in alpha-1-proteinase inhibitor-deficient lung transplant recipients

1 reference

12 December 2020

Pulmonary deposition and clearance of aerosolized alpha-1-proteinase inhibitor administered to dogs and to sheep

1 reference

12 December 2020

Pilot detection study of alpha(1) antitrypsin deficiency in a targeted population

1 reference

12 December 2020

Does alpha1-antitrypsin augmentation therapy slow the annual decline in FEV1 in patients with severe hereditary alpha1-antitrypsin deficiency? Wissenschaftliche Arbeitsgemeinschaft zur Therapie von Lungenerkrankungen (WATL) alpha1-AT study group.

1 reference

12 December 2020

Screening for alpha1-Pi deficiency in patients with lung diseases

1 reference

12 December 2020

Orthotopic liver transplantation in children: two-year experience with 47 patients.

1 reference

12 December 2020

133 Xe ventilatory studies in 1-antitrypsin deficiency.

1 reference

12 December 2020

Defective intracellular transport and processing of CFTR is the molecular basis of most cystic fibrosis

1 reference

12 December 2020

10.1007/S00408-007-9009-Y

Identifiers

DOI

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16 August 2017

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