Will the real bile acid sulfotransferase please stand up? Identification of Sult2a8 as a major hepatic bile acid sulfonating enzyme in mice1 за авторством Paul A. Dawson, Kenneth D.R. Setchell

Отримати повний текст

Tandem mass spectrometry of serum cholestanoic (C27) acids – Typical concentration ranges and application to the study of peroxisomal biogenesis disorders за авторством Wujuan Zhang, Monica Narvaez Rivas, Kenneth D.R. Setchell

Отримати повний текст

Long-Term Cholic Acid Therapy in Zellweger Spectrum Disorders за авторством James E. Heubi, Kenneth D.R. Setchell, Kevin E. Bove

Отримати повний текст

Serum Bile Acid Profiling and Mixed Model Analysis Reveal Biomarkers Associated with Pruritus Reduction in Maralixibat-Treated Patients with BSEP Deficiency за авторством Xueheng Zhao, Wujuan Zhang, Pamela Vig, Cory Kostrub, Kenneth D. R. Setchell

Отримати повний текст

Dietary Isoflavone Aglycons from Soy Germ Pasta Improves Reproductive Performance of Aging Hens and Lowers Cholesterol Levels of Egg Yolk за авторством Kenneth D. R. Setchell, Evangelia Mourvaki, Carlo Clerici, Simona Mattioli, Gabriele Brecchia, Cesare Castellini

Отримати повний текст

Automated in vivo platform for the discovery of functional food treatments of hypercholesterolemia. за авторством Robert M Littleton, Kevin J Haworth, Hong Tang, Kenneth D R Setchell, Sandra Nelson, Jay R Hove

Отримати повний текст

Successful treatment of infantile oxysterol 7α-hydroxylase deficiency with oral chenodeoxycholic acid за авторством Yun-Ping Tang, Jing-Yu Gong, Kenneth D. R. Setchell, Wujuan Zhang, Jing Zhao, Jian-She Wang

Отримати повний текст

Substrate compositional variation with tissue/region and Gba1 mutations in mouse models--implications for Gaucher disease. за авторством Ying Sun, Wujuan Zhang, You-Hai Xu, Brian Quinn, Nupur Dasgupta, Benjamin Liou, Kenneth D R Setchell, Gregory A Grabowski

Отримати повний текст

Ubiquitous transgene expression of the glucosylceramide-synthesizing enzyme accelerates glucosylceramide accumulation and storage cells in a Gaucher disease mouse model. за авторством Sonya Barnes, You-Hai Xu, Wujuan Zhang, Benjamin Liou, Kenneth D R Setchell, Liming Bao, Gregory A Grabowski, Ying Sun

Отримати повний текст

Substrate Reduction Therapy Reverses Mitochondrial, mTOR, and Autophagy Alterations in a Cell Model of Gaucher Disease за авторством Yanyan Peng, Benjamin Liou, Yi Lin, Venette Fannin, Wujuan Zhang, Ricardo A. Feldman, Kenneth D. R. Setchell, Gregory A. Grabowski, Ying Sun

Отримати повний текст

Cholestasis impairs gut microbiota development and bile salt hydrolase activity in preterm neonates за авторством Lauren E. Lynch, Amy B. Hair, Krishnakant G. Soni, Heeju Yang, Laura A. Gollins, Monica Narvaez-Rivas, Kenneth D. R. Setchell, Geoffrey A. Preidis

Отримати повний текст

Separation of micelles and vesicles within lumenal aspirates from healthy humans: solubilization of cholesterol after a meal за авторством Lihang Yao, James E. Heubi, Donna D. Buckley, Humberto Fierra, Kenneth D.R. Setchell, Norman A. Granholm, Patrick Tso, David Y. Hui, Laura A. Woollett

Отримати повний текст

Isofagomine in vivo effects in a neuronopathic Gaucher disease mouse. за авторством Ying Sun, Huimin Ran, Benjamin Liou, Brian Quinn, Matt Zamzow, Wujuan Zhang, Jacek Bielawski, Kazuyuki Kitatani, Kenneth D R Setchell, Yusuf A Hannun, Gregory A Grabowski

Отримати повний текст

Genetic spectrum and clinical characteristics of 3β-hydroxy-Δ5-C27-steroid oxidoreductase (HSD3B7) deficiency in China за авторством Jing Zhao, Kenneth D. R. Setchell, Ying Gong, Yinghua Sun, Ping Zhang, James E. Heubi, Lingjuan Fang, Yi Lu, Xinbao Xie, Jingyu Gong, Jian-She Wang

Отримати повний текст

Use of volumetric absorptive microsampling and parallel reaction monitoring mass spectrometry for tacrolimus blood trough measurements at home in pediatric heart transplant patient... за авторством Junfang Zhao, Kenneth D.R. Setchell, Xueheng Zhao, Stephanie Galandi, BreAnn N Garr, Zhiqian Gao, Clifford Chin, Shelly Stark, Paul E. Steele, Thomas D. Ryan

Отримати повний текст

Essential Fatty Acid Deficiency Associates with Growth Faltering and Environmental Enteric Dysfunction in Children за авторством Monica Narvaez-Rivas, Kenneth D. R. Setchell, Stephanie L. Galandi, Xueheng Zhao, Najeeha Talat Iqbal, Sheraz Ahmed, Junaid Iqbal, Sana Syed, Syed Asad Ali, Sean R. Moore

Отримати повний текст

Properties of neurons derived from induced pluripotent stem cells of Gaucher disease type 2 patient fibroblasts: potential role in neuropathology. за авторством Ying Sun, Jane Florer, Christopher N Mayhew, Zhanfeng Jia, Zhiying Zhao, Kui Xu, Huimin Ran, Benjamin Liou, Wujuan Zhang, Kenneth D R Setchell, Jianguo Gu, Gregory A Grabowski

Отримати повний текст

Progranulin Recruits HSP70 to β-Glucocerebrosidase and Is Therapeutic Against Gaucher Disease за авторством Jinlong Jian, Qing-Yun Tian, Aubryanna Hettinghouse, Shuai Zhao, Helen Liu, Jianlu Wei, Gabriele Grunig, Wujuan Zhang, Kenneth D.R. Setchell, Ying Sun, Herman S. Overkleeft, Gerald L. Chan, Chuan-ju Liu

Отримати повний текст

Maralixibat for the treatment of PFIC: Long‐term, IBAT inhibition in an open‐label, Phase 2 study за авторством Kathleen M. Loomes, Robert H. Squires, Deirdre Kelly, Sanjay Rajwal, Nisreen Soufi, Alain Lachaux, Irena Jankowska, Cara Mack, Kenneth D. R. Setchell, Palaniswamy Karthikeyan, Ciara Kennedy, Alejandro Dorenbaum, Nirav K. Desai, Will Garner, Thomas Jaecklin, Pamela Vig, Alexander Miethke, Richard J. Thompson

Отримати повний текст

Preventative treatment of tuberous sclerosis complex with sirolimus: Phase I safety and efficacy results за авторством Jamie K. Capal, David M. Ritter, David Neal Franz, Molly Griffith, Kristn Currans, Bridget Kent, E. Martina Bebin, Hope Northrup, Mary Kay Koenig, Tomoyuki Mizuno, Alexander A. Vinks, Stephanie L. Galandi, Wujuan Zhang, Kenneth D.R. Setchell, Kelly M. Kremer, Carlos M. Prada, Hansel M. Greiner, Katherine Holland‐Bouley, Paul S. Horn, Darcy A. Krueger

Отримати повний текст