Role of alpha-1 antitrypsin in human health and disease

Alpha-1 Antitrypsin Deficiency Targeted Testing and Augmentation Therapy: A Canadian Thoracic Society Clinical Practice Guideline

(2016) DD Marciniuk et al.   Canadian Respiratory Journal

α 1 -Antitrypsin Phenotypes and Associated Serum Protein Concentrations in a Large Clinical Population

(2013) Joshua A. Bornhorst et al.   CHEST

The Challenge of Detecting Alpha-1 Antitrypsin Deficiency

(2013) James K. Stoller et al.   COPD-Journal of Chronic Obstructive Pulmonary Disease

Alpha-1 Antitrypsin Deficiency: The European Experience

(2013) Robert A. Stockley et al.   COPD-Journal of Chronic Obstructive Pulmonary Disease

How Patients Learned to Control Their Own Future

(2013) John W. Walsh   COPD-Journal of Chronic Obstructive Pulmonary Disease

Inhaled Alpha 1-Antitrypsin: Gauging Patient Interest in a New Treatment

(2013) Richard Monk et al.   COPD-Journal of Chronic Obstructive Pulmonary Disease

Introduction to the 50th Anniversary of the Description of Alpha-1 Antitrypsin Deficiency

(2013) Robert A. Sandhaus et al.   COPD-Journal of Chronic Obstructive Pulmonary Disease

Gene-Based Therapy for Alpha-1 Antitrypsin Deficiency

(2013) Christian Mueller et al.   COPD-Journal of Chronic Obstructive Pulmonary Disease

Airway Disease in Alpha-1 Antitrypsin Deficiency

(2013) Charlie Strange   COPD-Journal of Chronic Obstructive Pulmonary Disease

Liver Disease in Alpha-1 Antitrypsin Deficiency: Current Understanding and Future Therapy

(2013) Jeffrey H. Teckman   COPD-Journal of Chronic Obstructive Pulmonary Disease

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

(2013) Elizabeth J. Carey et al.   LIVER TRANSPLANTATION

Anti-inflammatory and immunomodulatory properties of  1-antitrypsin without inhibition of elastase

(2013) D. Jonigk et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Novel Therapeutic Uses of Alpha-1 Antitrypsin: A Window to the Future

(2012) Adam Wanner et al.   COPD-Journal of Chronic Obstructive Pulmonary Disease

Alpha-1 antitrypsin deficiency PI*Z and PI*S gene frequency distribution using on maps of the world by an inverse distance weighting (IDW) multivariate interpolation method

(2012) Ignacio Blanco et al.   Hepatitis Monthly

Production and characterization of a novel human recombinant alpha-1-antitrypsin in PER.C6 cells

(2012) David Ross et al.   JOURNAL OF BIOTECHNOLOGY

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(2012) Eli Lewis   MOLECULAR MEDICINE

Fibromyalgia, mood disorders, and intense creative energy: A1AT polymorphisms are not always silent

(2012) Donald E. Schmechel et al.   NEUROTOXICOLOGY

Gene Therapy Meets Stem Cells

(2012) Robert A. Sandhaus   NEW ENGLAND JOURNAL OF MEDICINE

SERPINA1 PiZ and PiS Heterozygotes and Lung Function Decline in the SAPALDIA Cohort

(2012) Gian-Andri Thun et al.   PLoS One

Successful  1-antitrypsin replacement therapy in a patient with  1-antitrypsin deficiency and granulomatosis with polyangiitis

(2012) J. M. Hernandez Perez et al.   RHEUMATOLOGY

Alpha-1-antitrypsin augmentation therapy in deficient individuals enrolled in the Alpha-1 Foundation DNA and Tissue Bank

(2012) Adriano Tonelli   International Journal of Chronic Obstructive Pulmonary Disease

Reduction of severe exacerbations and hospitalization-derived costs in alpha-1-antitrypsin-deficient patients treated with alpha-1-antitrypsin augmentation therapy

(2012) Juan Carlos Barros-Tizón et al.   Therapeutic Advances in Respiratory Disease

Rare alpha-1-antitrypsin variants: are they really so rare?

(2012) Francisco Rodriguez-Frias et al.   Therapeutic Advances in Respiratory Disease

Prevalence of α1-antitrypsin deficiency alleles PI*S and PI*Z worldwide and effective screening for each of the five phenotypic classes PI*MS, PI*MZ, PI*SS, PI*SZ, and PI*ZZ: a comprehensive review

(2012) Frederick J. de Serres et al.   Therapeutic Advances in Respiratory Disease

A Review of α1-Antitrypsin Deficiency

(2011) James K. Stoller et al.   AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE

ANCA-associated vasculitis is linked to carriage of the Z allele of  1 antitrypsin and its polymers

(2011) H. Morris et al.   ANNALS OF THE RHEUMATIC DISEASES

Interleukin-1 in the pathogenesis and treatment of inflammatory diseases

(2011) C. A. Dinarello   BLOOD

Inhibition of IL-32 activation by  -1 antitrypsin suppresses alloreactivity and increases survival in an allogeneic murine marrow transplantation model

(2011) A. M. Marcondes et al.   BLOOD

Alpha-1 antitrypsin inhibits caspase-1 and protects from acute myocardial ischemia–reperfusion injury

(2011) Stefano Toldo et al.   JOURNAL OF MOLECULAR AND CELLULAR CARDIOLOGY

Severe ulcerative panniculitis caused by alpha 1-antitrypsin deficiency: Remission induced and maintained with intravenous alpha 1-antitrypsin

(2011) Firas Al-Niaimi et al.   JOURNAL OF THE AMERICAN ACADEMY OF DERMATOLOGY

Alpha-1 antitrypsin protein and gene therapies decrease autoimmunity and delay arthritis development in mouse model

(2011) Christian Grimstein et al.   Journal of Translational Medicine

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(2011) Galit Shahaf et al.   MOLECULAR MEDICINE

Targeted gene correction of α1-antitrypsin deficiency in induced pluripotent stem cells

(2011) Kosuke Yusa et al.   NATURE

Efficacy of alpha1-antitrypsin augmentation therapy in conditions other than pulmonary emphysema

(2011) Ignacio Blanco et al.   Orphanet Journal of Rare Diseases

Alpha-1-antitrypsin monotherapy reduces graft-versus-host disease after experimental allogeneic bone marrow transplantation

(2011) I. Tawara et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

The discovery of α1-antitrypsin and its role in health and disease

(2011) Sabina M. Janciauskiene et al.   RESPIRATORY MEDICINE

Oxidation of Z α1-Antitrypsin by Cigarette Smoke Induces Polymerization

(2010) Sam Alam et al.   AMERICAN JOURNAL OF RESPIRATORY CELL AND MOLECULAR BIOLOGY

Accelerated Spirometric Decline in New York City Firefighters With α; 1 -Antitrypsin Deficiency

(2010) Gisela I. Banauch et al.   CHEST

α; 1 -Antitrypsin Protease Inhibitor MZ Heterozygosity Is Associated With Airflow Obstruction in Two Large Cohorts

(2010) Inga-Cecilie Sørheim et al.   CHEST

Abnormal overexpression of mastocytes in skin biopsies of fibromyalgia patients

(2010) Ignacio Blanco et al.   CLINICAL RHEUMATOLOGY

Rapamycin reduces intrahepatic alpha-1-antitrypsin mutant Z protein polymers and liver injury in a mouse model

(2010) Shalesh Kaushal et al.   EXPERIMENTAL BIOLOGY AND MEDICINE

α-1 Antitrypsin regulates human neutrophil chemotaxis induced by soluble immune complexes and IL-8

(2010) David A. Bergin et al.   JOURNAL OF CLINICAL INVESTIGATION

Alpha1-Antitrypsin Deficiency and Autophagy

(2010) Stefan J. Marciniak et al.   NEW ENGLAND JOURNAL OF MEDICINE

Defining the mechanism of polymerization in the serpinopathies

(2010) U. I. Ekeowa et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

An Autophagy-Enhancing Drug Promotes Degradation of Mutant  1-Antitrypsin Z and Reduces Hepatic Fibrosis

(2010) T. Hidvegi et al.   SCIENCE

Short-Term Monotherapy in HIV-Infected Patients with a Virus Entry Inhibitor Against the gp41 Fusion Peptide

(2010) W.-G. Forssmann et al.   Science Translational Medicine

α 1-antitrypsin enhances insulin secretion and prevents cytokine-mediated apoptosis in pancreatic β-cells

(2010) Martins Kalis et al.   Islets

Ethnic differences in alpha-1 antitrypsin deficiency in the United States of America

(2010) Frederick J. de Serres et al.   Therapeutic Advances in Respiratory Disease

Augmentation Therapy for α1Antitrypsin Deficiency: A Meta-Analysis

(2009) Kenneth R. Chapman et al.   COPD-Journal of Chronic Obstructive Pulmonary Disease

Exploring the role of CT densitometry: a randomised study of augmentation therapy in  1-antitrypsin deficiency

(2009) A. Dirksen et al.   EUROPEAN RESPIRATORY JOURNAL

Safety/Efficacy of Inhaled Human Alpha-1 Antitrypsin (AAT) in CF: A Phase II Clinical Study

(2009) E. Kerem et al.   Journal of Cystic Fibrosis

Combination of alpha-1 antitrypsin and doxycycline suppresses collagen-induced arthritis

(2009) Christian Grimstein et al.   JOURNAL OF GENE MEDICINE

α-1-Antitrypsin is an endogenous inhibitor of proinflammatory cytokine production in whole blood

(2009) Gregory B. Pott et al.   JOURNAL OF LEUKOCYTE BIOLOGY

Sustained transgene expression despite T lymphocyte responses in a clinical trial of rAAV1-AAT gene therapy

(2009) M. L. Brantly et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Lung function in 30-year-old alpha-1-antitrypsin-deficient individuals

(2009) Elisabeth Bernspång et al.   RESPIRATORY MEDICINE

IL10Polymorphisms Are Associated with Airflow Obstruction in Severe α1-Antitrypsin Deficiency

(2007) Dawn L. DeMeo et al.   AMERICAN JOURNAL OF RESPIRATORY CELL AND MOLECULAR BIOLOGY