about
Controversies in the Mechanism of Total Parenteral Nutrition Induced PathologyAFM Imaging Reveals Topographic Diversity of Wild Type and Z Variant Polymers of Human α1-Proteinase InhibitorA novel monoclonal antibody to characterize pathogenic polymers in liver disease associated with alpha1-antitrypsin deficiency.PiZ mouse liver accumulates polyubiquitin conjugates that associate with catalytically active 26S proteasomesActivating transcription factor 6 limits intracellular accumulation of mutant α(1)-antitrypsin Z and mitochondrial damage in hepatoma cells.Diagnosis and management of patients with α1-antitrypsin (A1AT) deficiency.Children with Chronic Hepatitis B in the United States and Canada.Oxidative stress contributes to liver damage in a murine model of alpha-1-antitrypsin deficiency.Alpha1-antitrypsin deficiency in childhood.Sustained knockdown of a disease-causing gene in patient-specific induced pluripotent stem cells using lentiviral vector-based gene therapy.Rapamycin reduces intrahepatic alpha-1-antitrypsin mutant Z protein polymers and liver injury in a mouse modelAntisense oligonucleotide treatment ameliorates alpha-1 antitrypsin-related liver disease in mice.Appropriateness of newborn screening for α1-antitrypsin deficiency.Liver disease in alpha-1 antitrypsin deficiency: current understanding and future therapy.Advances in alpha-1-antitrypsin deficiency liver disease.Alpha-1 antitrypsin and liver disease: mechanisms of injury and novel interventions.Determination of hepatitis B phenotype using biochemical and serological markers.The Diagnosis and Management of Alpha-1 Antitrypsin Deficiency in the Adult.Activation of the c-Jun N-terminal kinase pathway aggravates proteotoxicity of hepatic mutant Z alpha1-antitrypsin.The serpinopathies studying serpin polymerization in vivo.In vivo post-transcriptional gene silencing of alpha-1 antitrypsin by adeno-associated virus vectors expressing siRNA.Alpha-1-antitrypsin mutant Z protein content in individual hepatocytes correlates with cell death in a mouse model.Health-related Quality of Life in Pediatric Patients With Chronic Hepatitis B Living in the United States and Canada.Conceptual advances in the pathogenesis and treatment of childhood metabolic liver disease.Gene transfer of master autophagy regulator TFEB results in clearance of toxic protein and correction of hepatic disease in alpha-1-anti-trypsin deficiency.Clinical Trial Design for Alpha-1 Antitrypsin Deficiency: A Model for Rare Diseases.Sustained miRNA-mediated knockdown of mutant AAT with simultaneous augmentation of wild-type AAT has minimal effect on global liver miRNA profiles.Corneal opacities associated with NTBC treatment.Obese children with steatohepatitis can develop cirrhosis in childhood.Mitochondrial autophagy and injury in the liver in alpha 1-antitrypsin deficiency.Preserved Gut Microbial Diversity Accompanies Upregulation of TGR5 and Hepatobiliary Transporters in Bile Acid-Treated Animals Receiving Parenteral Nutrition.Alpha-1-antitrypsin deficiency: Genetic variations, clinical manifestations and therapeutic interventions.Bone marrow stem cell therapy partially ameliorates pathological consequences in livers of mice expressing mutant human α1-antitrypsin.α1-Antitrypsin deficiency.Fasting in alpha1-antitrypsin deficient liver: constitutive [correction of consultative] activation of autophagy.Autophagy induced by exogenous bile acids is therapeutic in a model of α-1-AT deficiency liver disease.Oleanolic Acid Improves Gut Atrophy Induced by Parenteral Nutrition.Degradation of a mutant secretory protein, alpha1-antitrypsin Z, in the endoplasmic reticulum requires proteasome activityA naturally occurring nonpolymerogenic mutant of alpha 1-antitrypsin characterized by prolonged retention in the endoplasmic reticulumAlpha1-antitrypsin deficiency: from genotype to childhood disease
P50
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P50
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onderzoeker
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հետազոտող
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Jeffrey Teckman
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Jeffrey Teckman
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Jeffrey Teckman
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Jeffrey Teckman
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Jeffrey Teckman
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Jeffrey Teckman
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Jeffrey Teckman
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Jeffrey Teckman
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Jeffrey Teckman
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Jeffrey Teckman
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Jeffrey Teckman
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Jeffrey Teckman
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Jeffrey Teckman
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Jeffrey Teckman
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Jeffrey Teckman
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P106
P1153
6701751130
P31
P496
0000-0002-4870-2621