| Degradation of a mutant secretory protein, α1-antitrypsin Z, in the endoplasmic reticulum requires proteasome activity D Qu, JH Teckman, S Omura, DH Perlmutter Journal of Biological Chemistry 271 (37), 22791-22795, 1996 | 424 | 1996 |
| American Thoracic Society/European Respiratory Society statement: standards for the diagnosis and management of individuals with alpha-1 antitrypsin deficiency. JK Stoller, GL Snider, ML Brantly, RJ Fallat, RA Stockley, GM Turino, ... American Journal of Respiratory and Critical Care Medicine 168, 818-900, 2003 | 343 | 2003 |
| Retention of mutant α1-antitrypsin Z in endoplasmic reticulum is associated with an autophagic response JH Teckman, DH Perlmutter American Journal of Physiology-Gastrointestinal and Liver Physiology 279 (5 …, 2000 | 322 | 2000 |
| Obese children with steatohepatitis can develop cirrhosis in childhood JP Molleston, F White, J Teckman, JF Fitzgerald The American journal of gastroenterology 97 (9), 2460-2462, 2002 | 313 | 2002 |
| α1-Antitrypsin deficiency CM Greene, SJ Marciniak, J Teckman, I Ferrarotti, ML Brantly, DA Lomas, ... Nature reviews Disease primers 2 (1), 1-17, 2016 | 281 | 2016 |
| Mitochondrial autophagy and injury in the liver in α1-antitrypsin deficiency JH Teckman, JK An, K Blomenkamp, B Schmidt, D Perlmutter American Journal of Physiology-Gastrointestinal and Liver Physiology 286 (5 …, 2004 | 233 | 2004 |
| The diagnosis and management of alpha-1 antitrypsin deficiency in the adult RA Sandhaus, G Turino, ML Brantly, M Campos, CE Cross, K Goodman, ... Chronic Obstructive Pulmonary Diseases: Journal of the COPD Foundation 3 (3 …, 2016 | 214 | 2016 |
| A novel monoclonal antibody to characterize pathogenic polymers in liver disease associated with α1‐antitrypsin deficiency E Miranda, J Pérez, UI Ekeowa, N Hadzic, N Kalsheker, B Gooptu, ... Hepatology 52 (3), 1078-1088, 2010 | 171 | 2010 |
| Defining the mechanism of polymerization in the serpinopathies UI Ekeowa, J Freeke, E Miranda, B Gooptu, MF Bush, J Pérez, J Teckman, ... Proceedings of the National Academy of Sciences 107 (40), 17146-17151, 2010 | 157 | 2010 |
| Gene transfer of master autophagy regulator TFEB results in clearance of toxic protein and correction of hepatic disease in alpha‐1‐anti‐trypsin deficiency N Pastore, K Blomenkamp, F Annunziata, P Piccolo, P Mithbaokar, ... EMBO molecular medicine 5 (3), 397-412, 2013 | 154 | 2013 |
| Molecular pathogenesis of liver disease in α1‐antitrypsin deficiency JH Teckman, D Qu, DH Perlmutter Hepatology 24 (6), 1504-1516, 1996 | 150 | 1996 |
| Analyses of hepatocellular proliferation in a mouse model of α‐1‐antitrypsin deficiency DA Rudnick, Y Liao, JK An, LJ Muglia, DH Perlmutter, JH Teckman Hepatology 39 (4), 1048-1055, 2004 | 145 | 2004 |
| The proteasome participates in degradation of mutant α1-antitrypsin Z in the endoplasmic reticulum of hepatoma-derived hepatocytes JH Teckman, J Burrows, T Hidvegi, B Schmidt, PD Hale, DH Perlmutter Journal of Biological Chemistry 276 (48), 44865-44872, 2001 | 143 | 2001 |
| Total serum bilirubin within 3 months of hepatoportoenterostomy predicts short-term outcomes in biliary atresia BL Shneider, JC Magee, SJ Karpen, EB Rand, MR Narkewicz, LM Bass, ... The Journal of pediatrics 170, 211-217. e2, 2016 | 137 | 2016 |
| Rapamycin reduces intrahepatic alpha-1-antitrypsin mutant Z protein polymers and liver injury in a mouse model S Kaushal, M Annamali, K Blomenkamp, D Rudnick, D Halloran, EM Brunt, ... Experimental biology and medicine 235 (6), 700-709, 2010 | 133 | 2010 |
| Sustained miRNA-mediated knockdown of mutant AAT with simultaneous augmentation of wild-type AAT has minimal effect on global liver miRNA profiles C Mueller, Q Tang, A Gruntman, K Blomenkamp, J Teckman, L Song, ... Molecular Therapy 20 (3), 590-600, 2012 | 130 | 2012 |
| Lack of effect of oral 4-phenylbutyrate on serum alpha-1-antitrypsin in patients with α-1-antitrypsin deficiency: A preliminary study JH Teckman Journal of pediatric gastroenterology and nutrition 39 (1), 34-37, 2004 | 123 | 2004 |
| Fasting in α1-antitrypsin deficient liver: constitutive activation of autophagy JH Teckman, JK An, S Loethen, DH Perlmutter American Journal of Physiology-Gastrointestinal and Liver Physiology 283 (5 …, 2002 | 116 | 2002 |
| Diagnosis and management of patients with α1-antitrypsin (A1AT) deficiency DR Nelson, J Teckman, AM Di Bisceglie, DA Brenner Clinical Gastroenterology and Hepatology 10 (6), 575-580, 2012 | 112 | 2012 |
| The endoplasmic reticulum degradation pathway for mutant secretory proteins α1-antitrypsin Z and S is distinct from that for an unassembled membrane protein JH Teckman, DH Perlmutter Journal of Biological Chemistry 271 (22), 13215-13220, 1996 | 110 | 1996 |
