A 3D microfluidic liver model for high throughput compound toxicity screening in the OrganoPlate® KM Bircsak, R DeBiasio, M Miedel, A Alsebahi, R Reddinger, A Saleh, ... Toxicology 450, 152667, 2021 | 139 | 2021 |
Membrane traffic and turnover in TRP-ML1–deficient cells: a revised model for mucolipidosis type IV pathogenesis MT Miedel, Y Rbaibi, CJ Guerriero, G Colletti, KM Weixel, OA Weisz, ... The Journal of experimental medicine 205 (6), 1477-1490, 2008 | 107 | 2008 |
Posttranslational cleavage and adaptor protein complex-dependent trafficking of mucolipin-1 MT Miedel, KM Weixel, JR Bruns, LM Traub, OA Weisz Journal of Biological Chemistry 281 (18), 12751-12759, 2006 | 78 | 2006 |
Loss of lysosomal ion channel transient receptor potential channel mucolipin-1 (TRPML1) leads to cathepsin B-dependent apoptosis GA Colletti, MT Miedel, J Quinn, N Andharia, OA Weisz, K Kiselyov Journal of Biological Chemistry 287 (11), 8082-8091, 2012 | 61 | 2012 |
A Pro-Cathepsin L Mutant Is a Luminal Substrate for Endoplasmic-Reticulum-Associated Degradation in C. elegans MT Miedel, NJ Graf, KE Stephen, OS Long, SC Pak, DH Perlmutter, ... PloS one 7 (7), e40145, 2012 | 47 | 2012 |
A genome-wide RNAi screen identifies potential drug targets in a C. elegans model of α1-antitrypsin deficiency LP O'Reilly, OS Long, MC Cobanoglu, JA Benson, CJ Luke, MT Miedel, ... Human molecular genetics 23 (19), 5123-5132, 2014 | 46 | 2014 |
A C. elegans model of human α1-antitrypsin deficiency links components of the RNAi pathway to misfolded protein turnover OS Long, JA Benson, JH Kwak, CJ Luke, SJ Gosai, LP O'Reilly, Y Wang, ... Human molecular genetics 23 (19), 5109-5122, 2014 | 40 | 2014 |
Clinically observed estrogen receptor alpha mutations within the ligand-binding domain confer distinguishable phenotypes S Jia, MT Miedel, M Ngo, R Hessenius, N Chen, P Wang, A Bahreini, Z Li, ... Oncology 94 (3), 176-189, 2018 | 25 | 2018 |
Activation of the Caenorhabditis elegans degenerin channel by shear stress requires the MEC-10 subunit S Shi, CJ Luke, MT Miedel, GA Silverman, TR Kleyman Journal of Biological Chemistry 291 (27), 14012-14022, 2016 | 21 | 2016 |
SERPINB12 is a slow-binding inhibitor of granzyme A and hepsin JZ Niehaus, MT Miedel, M Good, AN Wyatt, SC Pak, GA Silverman, ... Biochemistry 54 (45), 6756-6759, 2015 | 19 | 2015 |
Modeling the effect of the metastatic microenvironment on phenotypes conferred by estrogen receptor mutations using a human liver microphysiological system MT Miedel, DC Gavlock, S Jia, A Gough, DL Taylor, AM Stern Scientific reports 9 (1), 8341, 2019 | 18 | 2019 |
Lysoptosis is an evolutionarily conserved cell death pathway moderated by intracellular serpins CJ Luke, S Markovina, M Good, IE Wight, BJ Thomas, JM Linneman, ... Communications Biology 5 (1), 47, 2022 | 17 | 2022 |
Deficient and Null Variants of SERPINA1 Are Proteotoxic in a Caenorhabditis elegans Model of α1-Antitrypsin Deficiency EE Cummings, LP O’Reilly, DE King, RM Silverman, MT Miedel, CJ Luke, ... PLoS One 10 (10), e0141542, 2015 | 13 | 2015 |
Quantifying the progression of non-alcoholic fatty liver disease in human biomimetic liver microphysiology systems with fluorescent protein biosensors M Saydmohammed, A Jha, V Mahajan, D Gavlock, TY Shun, R DeBiasio, ... Experimental Biology and Medicine 246 (22), 2420-2441, 2021 | 11 | 2021 |
ADP-ribosylation factor 1-independent protein sorting and export from the trans-Golgi network MA Ellis, MT Miedel, CJ Guerriero, OA Weisz Journal of Biological Chemistry 279 (50), 52735-52743, 2004 | 10 | 2004 |
A Quantitative Systems Pharmacology Platform Reveals NAFLD Pathophysiological States and Targeting Strategies DE Lefever, MT Miedel, F Pei, JK DiStefano, R Debiasio, TY Shun, ... Metabolites 12 (6), 528, 2022 | 9 | 2022 |
Isolation of serpin-interacting proteins in C. elegans using protein affinity purification MT Miedel, X Zeng, NA Yates, GA Silverman, CJ Luke Methods 68 (3), 536-541, 2014 | 9 | 2014 |
Using C. elegans to identify the protease targets of serpins in vivo SR Bhatia, MT Miedel, CK Chotoo, NJ Graf, BL Hood, TP Conrads, ... Methods in enzymology 499, 283-299, 2011 | 8 | 2011 |
The Combination of a Human Biomimetic Liver Microphysiology System with BIOLOGXsym, a Quantitative Systems Toxicology (QST) Modeling Platform for Macromolecules, Provides … JJ Beaudoin, L Clemens, MT Miedel, A Gough, F Zaidi, P Ramamoorthy, ... International Journal of Molecular Sciences 24 (11), 9692, 2023 | 6 | 2023 |
Polymorphisms associated with metabolic dysfunction-associated steatotic liver disease influence the progression of end-stage liver disease ZN Kocas-Kilicarslan, Z Cetin, LAP Faccioli, T Motomura, S Amirneni, ... Gastro hep advances 3 (1), 67-77, 2024 | 5 | 2024 |