Accumulation of mutant α1-antitrypsin Z in the endoplasmic reticulum activates caspases-4 and-12, NFκB, and BAP31 but not the unfolded protein response T Hidvegi, BZ Schmidt, P Hale, DH Perlmutter Journal of Biological Chemistry 280 (47), 39002-39015, 2005 | 266 | 2005 |
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 |
Human factor H deficiency BH Ault, BZ Schmidt, NL Fowler, CE Kashtan, AE Ahmed, BA Vogt, ... Journal of Biological Chemistry 272 (40), 25168-25175, 1997 | 195 | 1997 |
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 |
In vitro acute and developmental neurotoxicity screening: an overview of cellular platforms and high-throughput technical possibilities BZ Schmidt, M Lehmann, S Gutbier, E Nembo, S Noel, L Smirnova, ... Archives of toxicology 91, 1-33, 2017 | 142 | 2017 |
Efficient recombinase-mediated cassette exchange in hPSCs to study the hepatocyte lineage reveals AAVS1 locus-mediated transgene inhibition L Ordovás, R Boon, M Pistoni, Y Chen, E Wolfs, W Guo, R Sambathkumar, ... Stem cell reports 5 (5), 918-931, 2015 | 134 | 2015 |
Grp78, Grp94, and Grp170 interact with α1-antitrypsin mutants that are retained in the endoplasmic reticulum BZ Schmidt, DH Perlmutter American Journal of Physiology-Gastrointestinal and Liver Physiology 289 (3 …, 2005 | 123 | 2005 |
Small heat shock proteins target mutant cystic fibrosis transmembrane conductance regulator for degradation via a small ubiquitin-like modifier–dependent pathway A Ahner, X Gong, BZ Schmidt, KW Peters, WM Rabeh, PH Thibodeau, ... Molecular biology of the cell 24 (2), 74-84, 2013 | 111 | 2013 |
Cystic fibrosis transmembrane conductance regulator modulators in cystic fibrosis: current perspectives BZ Schmidt, JB Haaf, T Leal, S Noel Clinical pharmacology: advances and applications, 127-140, 2016 | 80 | 2016 |
A naturally occurring nonpolymerogenic mutant of α1-antitrypsin characterized by prolonged retention in the endoplasmic reticulum L Lin, B Schmidt, J Teckman, DH Perlmutter Journal of Biological Chemistry 276 (36), 33893-33898, 2001 | 70 | 2001 |
Cysteine string protein monitors late steps in cystic fibrosis transmembrane conductance regulator biogenesis H Zhang, BZ Schmidt, F Sun, SB Condliffe, MB Butterworth, RT Youker, ... Journal of Biological Chemistry 281 (16), 11312-11321, 2006 | 66 | 2006 |
Cysteine string protein promotes proteasomal degradation of the cystic fibrosis transmembrane conductance regulator (CFTR) by increasing its interaction with the C terminus of … BZ Schmidt, RJ Watts, M Aridor, RA Frizzell Journal of Biological Chemistry 284 (7), 4168-4178, 2009 | 56 | 2009 |
Prdm12 directs nociceptive sensory neuron development by regulating the expression of the NGF receptor TrkA S Desiderio, S Vermeiren, C Van Campenhout, S Kricha, E Malki, ... Cell reports 26 (13), 3522-3536. e5, 2019 | 54 | 2019 |
Disruption of disulfide bonds is responsible for impaired secretion in human complement factor H deficiency BZ Schmidt, NL Fowler, T Hidvegi, DH Perlmutter, HR Colten Journal of Biological Chemistry 274 (17), 11782-11788, 1999 | 54 | 1999 |
Complement: a critical test of its biological importance. BZ Schmidt, HR Colten Immunological reviews 178 (1), 2000 | 46 | 2000 |
The EU-ToxRisk method documentation, data processing and chemical testing pipeline for the regulatory use of new approach methods A Krebs, BMA van Vugt-Lussenburg, T Waldmann, W Albrecht, J Boei, ... Archives of Toxicology 94, 2435-2461, 2020 | 41 | 2020 |
Nanomaterials to avoid and destroy protein aggregates F Sauvage, J Schymkowitz, F Rousseau, BZ Schmidt, K Remaut, ... Nano Today 31, 100837, 2020 | 32 | 2020 |
ADD66, a Gene Involved in the Endoplasmic Reticulum-associated Degradation of α-1-Antitrypsin-Z in Yeast, Facilitates Proteasome Activity and Assembly CM Scott, KB Kruse, BZ Schmidt, DH Perlmutter, AA McCracken, ... Molecular biology of the cell 18 (10), 3776-3787, 2007 | 32 | 2007 |
Accumulation of mutant alpha 1 antitrypsin Z in the ER activates caspases-4 and-12, NFkappa B and BAP31 but not the unfolded protein response T Hidvegi, BZ Schmidt, P Hale, DH Perlmutter J Biol Chem 280 (47), 39002-15, 2005 | 28 | 2005 |
Determination of benchmark concentrations and their statistical uncertainty for cytotoxicity test data and functional in vitro assays A Krebs, J Nyffeler, C Karreman, BZ Schmidt, F Kappenberg, J Mellert, ... | 26 | 2020 |