List of works - Jeffrey Teckman

A naturally occurring nonpolymerogenic mutant of alpha 1-antitrypsin characterized by prolonged retention in the endoplasmic reticulum

scientific article published on 26 June 2001

A novel monoclonal antibody to characterize pathogenic polymers in liver disease associated with alpha1-antitrypsin deficiency.

scientific article

AFM Imaging Reveals Topographic Diversity of Wild Type and Z Variant Polymers of Human α1-Proteinase Inhibitor

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

Activation of the c-Jun N-terminal kinase pathway aggravates proteotoxicity of hepatic mutant Z alpha1-antitrypsin

scientific article published on 10 January 2017

Advances in alpha-1-antitrypsin deficiency liver disease

scientific article published on January 2014

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

scientific article

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

scientific article published on 18 March 2017

Alpha-1-antitrypsin mutant Z protein content in individual hepatocytes correlates with cell death in a mouse model.

scientific article published on October 2007

Alpha1-antitrypsin deficiency in childhood.

scientific article published on August 2007

Alpha1-antitrypsin deficiency: from genotype to childhood disease

scientific article published on 01 July 1998

Antisense oligonucleotide treatment ameliorates alpha-1 antitrypsin-related liver disease in mice.

scientific article published on 20 December 2013

Appropriateness of newborn screening for α1-antitrypsin deficiency

scientific article published on February 2014

Author's Response

Author's response

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

Bone marrow stem cell therapy partially ameliorates pathological consequences in livers of mice expressing mutant human α1-antitrypsin

scientific article

Children with Chronic Hepatitis B in the United States and Canada

scientific article

Clinical Trial Design for Alpha-1 Antitrypsin Deficiency: A Model for Rare Diseases

scientific article published on 28 April 2015

Conceptual advances in the pathogenesis and treatment of childhood metabolic liver disease.

scientific article

Controversies in the Mechanism of Total Parenteral Nutrition Induced Pathology

scientific article

Corneal opacities associated with NTBC treatment.

scientific article

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

scientific article published on 01 September 1996

Determination of hepatitis B phenotype using biochemical and serological markers.

scientific article published on 05 December 2016

Diagnosis and management of patients with α1-antitrypsin (A1AT) deficiency

scientific article

Emerging Concepts and Human Trials in Alpha-1-Antitrypsin Deficiency Liver Disease

scientific article published on 31 May 2017

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

scientific article published in November 2002

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

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

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

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

Liver disease in alpha-1 antitrypsin deficiency: current understanding and future therapy

scientific article published on March 2013

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

scientific article published on 18 December 2003

NorUDCA promotes degradation of α1-antitrypsin mutant Z protein by inducing autophagy through AMPK/ULK1 pathway

scientific article published on 01 August 2018

Obese children with steatohepatitis can develop cirrhosis in childhood

scientific article published in September 2002

Oleanolic Acid Improves Gut Atrophy Induced by Parenteral Nutrition.

scientific article

Oxidative stress contributes to liver damage in a murine model of alpha-1-antitrypsin deficiency.

scientific article

Pediatric Intestinal Failure Review

scientific article published on 20 July 2018

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

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

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

scientific article published on June 2010

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

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

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

The Diagnosis and Management of Alpha-1 Antitrypsin Deficiency in the Adult

scientific article published on 06 June 2016

The serpinopathies studying serpin polymerization in vivo

scientific article

Total serum bilirubin predicts fat-soluble vitamin deficiency better than serum bile acids in infants with biliary atresia

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

α1-Antitrypsin deficiency.

scientific article published on 28 July 2016

List of works by Jeffrey Teckman

A naturally occurring nonpolymerogenic mutant of alpha 1-antitrypsin characterized by prolonged retention in the endoplasmic reticulum

A novel monoclonal antibody to characterize pathogenic polymers in liver disease associated with alpha1-antitrypsin deficiency.

AFM Imaging Reveals Topographic Diversity of Wild Type and Z Variant Polymers of Human α1-Proteinase Inhibitor

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

Activation of the c-Jun N-terminal kinase pathway aggravates proteotoxicity of hepatic mutant Z alpha1-antitrypsin

Advances in alpha-1-antitrypsin deficiency liver disease

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

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

Alpha-1-antitrypsin mutant Z protein content in individual hepatocytes correlates with cell death in a mouse model.

Alpha1-antitrypsin deficiency in childhood.

Alpha1-antitrypsin deficiency: from genotype to childhood disease

Antisense oligonucleotide treatment ameliorates alpha-1 antitrypsin-related liver disease in mice.

Appropriateness of newborn screening for α1-antitrypsin deficiency

Author's Response

Author's response

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

Bone marrow stem cell therapy partially ameliorates pathological consequences in livers of mice expressing mutant human α1-antitrypsin

Children with Chronic Hepatitis B in the United States and Canada

Clinical Trial Design for Alpha-1 Antitrypsin Deficiency: A Model for Rare Diseases

Conceptual advances in the pathogenesis and treatment of childhood metabolic liver disease.

Controversies in the Mechanism of Total Parenteral Nutrition Induced Pathology

Corneal opacities associated with NTBC treatment.

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

Determination of hepatitis B phenotype using biochemical and serological markers.

Diagnosis and management of patients with α1-antitrypsin (A1AT) deficiency

Emerging Concepts and Human Trials in Alpha-1-Antitrypsin Deficiency Liver Disease

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

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

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

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

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

Liver disease in alpha-1 antitrypsin deficiency: current understanding and future therapy

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

NorUDCA promotes degradation of α1-antitrypsin mutant Z protein by inducing autophagy through AMPK/ULK1 pathway

Obese children with steatohepatitis can develop cirrhosis in childhood

Oleanolic Acid Improves Gut Atrophy Induced by Parenteral Nutrition.

Oxidative stress contributes to liver damage in a murine model of alpha-1-antitrypsin deficiency.

Pediatric Intestinal Failure Review

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

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

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

Selected summary. Extrahepatic biliary atresia (EHBA) is still a significant medical and economic burden to patients and families

Sustained knockdown of a disease-causing gene in patient-specific induced pluripotent stem cells using lentiviral vector-based gene therapy

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

The Diagnosis and Management of Alpha-1 Antitrypsin Deficiency in the Adult

The serpinopathies studying serpin polymerization in vivo

Total serum bilirubin predicts fat-soluble vitamin deficiency better than serum bile acids in infants with biliary atresia

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

α1-Antitrypsin deficiency.