Search Results - "Miedel, Mark T"

The Combination of a Human Biomimetic Liver Microphysiology System with BIOLOGXsym, a Quantitative Systems Toxicology (QST) Modeling Platform for Macromolecules, Provides Mechanistic Understanding of Tocilizumab- and GGF2-Induced Liver Injury by Beaudoin, James J, Clemens, Lara, Miedel, Mark T, Gough, Albert, Zaidi, Fatima, Ramamoorthy, Priya, Wong, Kari E, Sarangarajan, Rangaprasad, Battista, Christina, Shoda, Lisl K M, Siler, Scott Q, Taylor, D Lansing, Howell, Brett A, Vernetti, Lawrence A, Yang, Kyunghee

“…Biologics address a range of unmet clinical needs, but the occurrence of biologics-induced liver injury remains a major challenge. Development of cimaglermin…”
Get full text

Evaluation of Human Hepatocyte Drug Metabolism Carrying High-Risk or Protection-Associated Liver Disease Genetic Variants by Faccioli, Lanuza A P, Cetin, Zeliha, Kocas-Kilicarslan, Zehra N, Ortiz, Kimberly, Sun, Yiyue, Hu, Zhiping, Kurihara, Takeshi, Tafaleng, Edgar N, Florentino, Rodrigo M, Wang, Zi, Xia, Mengying, Miedel, Mark T, Taylor, D Lansing, Behari, Jaideep, Ostrowska, Alina, Constantine, Robert, Li, Albert, Soto-Gutierrez, Alejandro

“…Metabolic-dysfunction-associated steatotic liver disease (MASLD), which affects 30 million people in the US and is anticipated to reach over 100 million by…”
Get full text

A pro-cathepsin L mutant is a luminal substrate for endoplasmic-reticulum-associated degradation in C. elegans by Miedel, Mark T, Graf, Nathan J, Stephen, Kate E, Long, Olivia S, Pak, Stephen C, Perlmutter, David H, Silverman, Gary A, Luke, Cliff J

“…Endoplasmic-reticulum associated degradation (ERAD) is a major cellular misfolded protein disposal pathway that is well conserved from yeast to mammals. In…”
Get full text

A metabolic dysfunction-associated steatotic liver acinus biomimetic induces pancreatic islet dysfunction in a coupled microphysiology system by Aleman, Julio, K, Ravikumar, Wiegand, Connor, Schurdak, Mark E., Vernetti, Lawrence, Gavlock, Dillon, Reese, Celeste, DeBiasio, Richard, LaRocca, Greg, Angarita, Yulder Daniel, Gough, Albert, Soto-Gutierrez, Alejandro, Behari, Jaideep, Yechoor, Vijay K., Miedel, Mark T., Stern, Andrew M., Banerjee, Ipsita, Taylor, D. Lansing

“…Preclinical and clinical studies suggest that lipid-induced hepatic insulin resistance is a primary defect that predisposes to dysfunction in islets,…”
Get full text

Comparison of wild-type and high-risk PNPLA3 variants in a human biomimetic liver microphysiology system for metabolic dysfunction-associated steatotic liver disease precision therapy by Xia, Mengying, Varmazyad, Mahboubeh, Pla-Palacín, Iris, Gavlock, Dillon C, DeBiasio, Richard, LaRocca, Gregory, Reese, Celeste, Florentino, Rodrigo M, Faccioli, Lanuza A P, Brown, Jacquelyn A, Vernetti, Lawrence A, Schurdak, Mark, Stern, Andrew M, Gough, Albert, Behari, Jaideep, Soto-Gutierrez, Alejandro, Taylor, D Lansing, Miedel, Mark T

“…Metabolic dysfunction-associated steatotic liver disease (MASLD) is a worldwide health epidemic with a global occurrence of approximately 30%. The pathogenesis…”
Get full text

Polymorphisms Associated With Metabolic Dysfunction-Associated Steatotic Liver Disease Influence the Progression of End-Stage Liver Disease by Kocas-Kilicarslan, Zehra N., Cetin, Zeliha, Faccioli, Lanuza A.P., Motomura, Takashi, Amirneni, Sriram, Diaz-Aragon, Ricardo, Florentino, Rodrigo M., Sun, Yiyue, Pla-Palacin, Iris, Xia, Mengying, Miedel, Mark T., Kurihara, Takeshi, Hu, Zhiping, Ostrowska, Alina, Wang, Zi, Constantine, Robert, Li, Albert, Taylor, D. Lansing, Behari, Jaideep, Soto-Gutierrez, Alejandro, Tafaleng, Edgar N.

“…Chronic liver injury that results in cirrhosis and end-stage liver disease (ESLD) causes more than 1 million deaths annually worldwide. Although the impact of…”
Get full text

Deficient and Null Variants of SERPINA1 Are Proteotoxic in a Caenorhabditis elegans Model of α1-Antitrypsin Deficiency by Cummings, Erin E, O'Reilly, Linda P, King, Dale E, Silverman, Richard M, Miedel, Mark T, Luke, Cliff J, Perlmutter, David H, Silverman, Gary A, Pak, Stephen C

“…α1-antitrypsin deficiency (ATD) predisposes patients to both loss-of-function (emphysema) and gain-of-function (liver cirrhosis) phenotypes depending on the…”
Get full text

A Quantitative Systems Pharmacology Platform Reveals NAFLD Pathophysiological States and Targeting Strategies by Lefever, Daniel E, Miedel, Mark T, Pei, Fen, DiStefano, Johanna K, Debiasio, Richard, Shun, Tong Ying, Saydmohammed, Manush, Chikina, Maria, Vernetti, Lawrence A, Soto-Gutierrez, Alejandro, Monga, Satdarshan P, Bataller, Ramon, Behari, Jaideep, Yechoor, Vijay K, Bahar, Ivet, Gough, Albert, Stern, Andrew M, Taylor, D Lansing

“…Non-alcoholic fatty liver disease (NAFLD) has a high global prevalence with a heterogeneous and complex pathophysiology that presents barriers to traditional…”
Get full text

Lysoptosis is an evolutionarily conserved cell death pathway moderated by intracellular serpins by Luke, Cliff J., Markovina, Stephanie, Good, Misty, Wight, Ira E., Thomas, Brian J., Linneman, John M., Lanik, Wyatt E., Koroleva, Olga, Coffman, Maggie R., Miedel, Mark T., Gong, Qingqing, Andress, Arlise, Campos Guerrero, Marlene, Wang, Songyan, Chen, LiYun, Beatty, Wandy L., Hausmann, Kelsey N., White, Frances V., Fitzpatrick, James A. J., Orvedahl, Anthony, Pak, Stephen C., Silverman, Gary A.

“…Lysosomal membrane permeabilization (LMP) and cathepsin release typifies lysosome-dependent cell death (LDCD). However, LMP occurs in most regulated cell death…”
Get full text

Posttranslational Cleavage and Adaptor Protein Complex-dependent Trafficking of Mucolipin-1 by Miedel, Mark T., Weixel, Kelly M., Bruns, Jennifer R., Traub, Linton M., Weisz, Ora A.

“…Mucolipin-1 (ML1) is a member of the transient receptor potential ion channel superfamily that is thought to function in the biogenesis of lysosomes. Mutations…”
Get full text

831-P: Tocilizumab Is Effective in Reducing Inflammation in T2D–COVID-Organ-on-a-Chip Model by NEGI, VINNY, GAVLOCK, DILLON, LEE, JEONGKYUNG, SHUN, TONGYING, GOUGH, ALBERT, MIEDEL, MARK T., TAYLOR, D. LANSING L., YECHOOR, VIJAY

“…The COVID-pandemic has contributed to more than 5 million deaths worldwide in the last two years. Co-morbid conditions such as Type 2 Diabetes (T2D) , HTN,…”
Get full text

Human induced pluripotent stem cell based hepatic-modeling of lipid metabolism associated TM6SF2 E167K variant by Faccioli, Lanuza Ap, Sun, Yiyue, Animasahun, Olamide, Motomura, Takashi, Liu, Zhenghao, Kurihara, Takeshi, Hu, Zhiping, Yang, Bo, Cetin, Zeliha, Baratta, Annalisa M, Shankaran, Ajay, Nenwani, Minal, Altay, Leyla Nurcihan, Huang, Linqi, Meurs, Noah, Franks, Jonathan, Stolz, Donna, Gavlock, Dillon C, Miedel, Mark T, Ostrowska, Alina, Florentino, Rodrigo M, Fox, Ira J, Nagrath, Deepak, Soto-Gutierrez, Alejandro

“…TM6SF2 rs58542926 (E167K) is related to increased prevalence of metabolic dysfunction-associated steatotic liver disease (MASLD). Conflicting mouse study…”
Get full text

Quantifying the progression of non-alcoholic fatty liver disease in human biomimetic liver microphysiology systems with fluorescent protein biosensors by Saydmohammed, Manush, Jha, Anupma, Mahajan, Vineet, Gavlock, Dillon, Shun, Tong Ying, DeBiasio, Richard, Lefever, Daniel, Li, Xiang, Reese, Celeste, Kershaw, Erin E, Yechoor, Vijay, Behari, Jaideep, Soto-Gutierrez, Alejandro, Vernetti, Larry, Stern, Andrew, Gough, Albert, Miedel, Mark T, Lansing Taylor, D

“…Metabolic syndrome is a complex disease that involves multiple organ systems including a critical role for the liver. Non-alcoholic fatty liver disease (NAFLD)…”
Get full text

Modeling the Effect of the Metastatic Microenvironment on Phenotypes Conferred by Estrogen Receptor Mutations Using a Human Liver Microphysiological System by Miedel, Mark T., Gavlock, Dillon C., Jia, Shanhang, Gough, Albert, Taylor, D. Lansing, Stern, Andrew M.

“…Reciprocal coevolution of tumors and their microenvironments underlies disease progression, yet intrinsic limitations of patient-derived xenografts and simpler…”
Get full text

Activation of the Caenorhabditis elegans Degenerin Channel by Shear Stress Requires the MEC-10 Subunit by Shi, Shujie, Luke, Cliff J., Miedel, Mark T., Silverman, Gary A., Kleyman, Thomas R.

“…Mechanotransduction in Caenorhabditis elegans touch receptor neurons is mediated by an ion channel formed by MEC-4, MEC-10, and accessory proteins. To define…”
Get full text

Loss of Lysosomal Ion Channel Transient Receptor Potential Channel Mucolipin-1 (TRPML1) Leads to Cathepsin B-dependent Apoptosis by Colletti, Grace A., Miedel, Mark T., Quinn, James, Andharia, Neel, Weisz, Ora A., Kiselyov, Kirill

“…Mucolipidosis type IV (MLIV) is a lysosomal storage disease caused by mutations in the gene MCOLN1, which codes for the transient receptor potential family ion…”
Get full text

A genome-wide RNAi screen identifies potential drug targets in a C. elegans model of α1-antitrypsin deficiency by O'Reilly, Linda P, Long, Olivia S, Cobanoglu, Murat C, Benson, Joshua A, Luke, Cliff J, Miedel, Mark T, Hale, Pamela, Perlmutter, David H, Bahar, Ivet, Silverman, Gary A, Pak, Stephen C

“…α1-Antitrypsin deficiency (ATD) is a common genetic disorder that can lead to end-stage liver and lung disease. Although liver transplantation remains the only…”
Get full text

SERPINB12 Is a Slow-Binding Inhibitor of Granzyme A and Hepsin by Niehaus, Jason Z, Miedel, Mark T, Good, Misty, Wyatt, Allyson N, Pak, Stephen C, Silverman, Gary A, Luke, Cliff J

“…The clade B/intracellular serpins protect cells from peptidase-mediated injury by forming covalent complexes with their targets. SERPINB12 is expressed in most…”
Get full text

Clinically Observed Estrogen Receptor Alpha Mutations within the Ligand-Binding Domain Confer Distinguishable Phenotypes by Jia, Shanhang, Miedel, Mark T, Ngo, Marilyn, Hessenius, Ryan, Chen, Ning, Wang, Peilu, Bahreini, Amir, Li, Zheqi, Ding, Zhijie, Shun, Tong Ying, Zuckerman, Daniel M, Taylor, D Lansing, Puhalla, Shannon L, Lee, Adrian V, Oesterreich, Steffi, Stern, Andrew M

“…Twenty to fifty percent of estrogen receptor-positive (ER+) metastatic breast cancers express mutations within the ER ligand-binding domain. While most studies…”
Get more information

Membrane traffic and turnover in TRP-ML1-deficient cells: a revised model for mucolipidosis type IV pathogenesis by Miedel, Mark T, Rbaibi, Youssef, Guerriero, Christopher J, Colletti, Grace, Weixel, Kelly M, Weisz, Ora A, Kiselyov, Kirill

“…The lysosomal storage disorder mucolipidosis type IV (MLIV) is caused by mutations in the transient receptor potential-mucolipin-1 (TRP-ML1) ion channel. The…”
Get full text