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List of works by Jeffrey Teckman

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

scientific article published on 01 September 1996
Mitochondrial autophagy and injury in the liver in alpha 1-antitrypsin deficiency.

scientific article published on 18 December 2003
Gene transfer of master autophagy regulator TFEB results in clearance of toxic protein and correction of hepatic disease in alpha-1-anti-trypsin deficiency

scientific article published on 4 February 2013
Obese children with steatohepatitis can develop cirrhosis in childhood

scientific article published in September 2002
A novel monoclonal antibody to characterize pathogenic polymers in liver disease associated with alpha1-antitrypsin deficiency.

scientific article
Sustained miRNA-mediated knockdown of mutant AAT with simultaneous augmentation of wild-type AAT has minimal effect on global liver miRNA profiles.

scientific article published on 17 January 2012
Fasting in alpha1-antitrypsin deficient liver: constitutive [correction of consultative] activation of autophagy

scientific article published in November 2002
Rapamycin reduces intrahepatic alpha-1-antitrypsin mutant Z protein polymers and liver injury in a mouse model

scientific article published on June 2010
The Diagnosis and Management of Alpha-1 Antitrypsin Deficiency in the Adult

scientific article published on 06 June 2016
Alpha-1-antitrypsin mutant Z protein content in individual hepatocytes correlates with cell death in a mouse model.

scientific article published on October 2007
α1-Antitrypsin deficiency.

scientific article published on 28 July 2016
Diagnosis and management of patients with α1-antitrypsin (A1AT) deficiency

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

scientific article
Activating transcription factor 6 limits intracellular accumulation of mutant α(1)-antitrypsin Z and mitochondrial damage in hepatoma cells.

scientific article published on 5 October 2011
Liver Disease in Alpha-1 Antitrypsin Deficiency: Current Understanding and Future Therapy

scientific article published on March 1, 2013
Antisense oligonucleotide treatment ameliorates alpha-1 antitrypsin-related liver disease in mice.

scientific article published on 20 December 2013
A naturally occurring nonpolymerogenic mutant of alpha 1-antitrypsin characterized by prolonged retention in the endoplasmic reticulum

scientific article published on 26 June 2001
Oxidative stress contributes to liver damage in a murine model of alpha-1-antitrypsin deficiency.

scientific article
Indomethacin increases liver damage in a murine model of liver injury from alpha-1-antitrypsin deficiency

scientific article published on 01 October 2006
Sustained knockdown of a disease-causing gene in patient-specific induced pluripotent stem cells using lentiviral vector-based gene therapy

scientific article published on 07 August 2013
Corneal opacities associated with NTBC treatment.

scientific article
The serpinopathies studying serpin polymerization in vivo

scientific article
Advances in alpha-1-antitrypsin deficiency liver disease

scientific article published on January 2014
Alpha1-antitrypsin deficiency in childhood.

scientific article published on August 2007
Appropriateness of newborn screening for α1-antitrypsin deficiency

scientific article published on February 2014
Conceptual advances in the pathogenesis and treatment of childhood metabolic liver disease.

scientific article
Alpha1-antitrypsin deficiency: from genotype to childhood disease

scientific article published on 01 July 1998
Bone marrow stem cell therapy partially ameliorates pathological consequences in livers of mice expressing mutant human α1-antitrypsin

scientific article
Alpha-1-antitrypsin deficiency: Genetic variations, clinical manifestations and therapeutic interventions.

scientific article published on 18 March 2017
Total serum bilirubin predicts fat-soluble vitamin deficiency better than serum bile acids in infants with biliary atresia

scientific article
Alpha-1 antitrypsin and liver disease: mechanisms of injury and novel interventions

scientific article
Preserved Gut Microbial Diversity Accompanies Upregulation of TGR5 and Hepatobiliary Transporters in Bile Acid-Treated Animals Receiving Parenteral Nutrition

scientific article published on 8 August 2016
Controversies in the Mechanism of Total Parenteral Nutrition Induced Pathology

scientific article
Autophagy induced by exogenous bile acids is therapeutic in a model of α-1-AT deficiency liver disease

scientific article published on 21 April 2016
Activation of the c-Jun N-terminal kinase pathway aggravates proteotoxicity of hepatic mutant Z alpha1-antitrypsin

scientific article published on 10 January 2017
Determination of hepatitis B phenotype using biochemical and serological markers.

scientific article published on 05 December 2016
Oleanolic Acid Improves Gut Atrophy Induced by Parenteral Nutrition

scientific article
Children with Chronic Hepatitis B in the United States and Canada

scientific article
Validating hyperbilirubinemia and gut mucosal atrophy with a novel ultramobile ambulatory total parenteral nutrition piglet model

scientific article published on 31 December 2014
AFM Imaging Reveals Topographic Diversity of Wild Type and Z Variant Polymers of Human α1-Proteinase Inhibitor

scientific article
Baseline Analysis of a Young α‐1‐Antitrypsin Deficiency Liver Disease Cohort Reveals Frequent Portal Hypertension

scientific article published on July 1, 2015
NorUDCA promotes degradation of α1-antitrypsin mutant Z protein by inducing autophagy through AMPK/ULK1 pathway

scientific article published on 01 August 2018
Clinical Trial Design for Alpha-1 Antitrypsin Deficiency: A Model for Rare Diseases

scientific article published on 28 April 2015
Emerging Concepts and Human Trials in Alpha-1-Antitrypsin Deficiency Liver Disease

scientific article published on 31 May 2017
Author's response

PiZ mouse liver accumulates polyubiquitin conjugates that associate with catalytically active 26S proteasomes

scientific article
Health-related Quality of Life in Pediatric Patients With Chronic Hepatitis B Living in the United States and Canada

scientific article published on 4 February 2017
Pediatric Intestinal Failure Review

scientific article published on 20 July 2018
Selected summary. Extrahepatic biliary atresia (EHBA) is still a significant medical and economic burden to patients and families

scientific article published on 01 January 2009
Author's Response

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