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

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

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

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

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

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

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Isofagomine in vivo effects in a neuronopathic Gaucher disease mouse. 著者: 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

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Use of volumetric absorptive microsampling and parallel reaction monitoring mass spectrometry for tacrolimus blood trough measurements at home in pediatric heart transplant patient... 著者: 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

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Essential Fatty Acid Deficiency Associates with Growth Faltering and Environmental Enteric Dysfunction in Children 著者: 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

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Maralixibat for the treatment of PFIC: Long‐term, IBAT inhibition in an open‐label, Phase 2 study 著者: 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

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Preventative treatment of tuberous sclerosis complex with sirolimus: Phase I safety and efficacy results 著者: 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

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Upregulation of acid ceramidase contributes to tumor progression in tuberous sclerosis complex 著者: Aristotelis Astrinidis, Chenggang Li, Erik Y. Zhang, Xueheng Zhao, Shuyang Zhao, Minzhe Guo, Tasnim Olatoke, Ushodaya Mattam, Rong Huang, Alan G. Zhang, Lori Pitstick, Elizabeth J. Kopras, Nishant Gupta, Roman Jandarov, Eric P. Smith, Elizabeth Fugate, Diana Lindquist, Maciej M. Markiewski, Magdalena Karbowniczek, Kathryn A. Wikenheiser-Brokamp, Kenneth D. R. Setchell, Francis X. McCormack, Yan Xu, Jane J. Yu

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Study of Environmental Enteropathy and Malnutrition (SEEM) in Pakistan: protocols for biopsy based biomarker discovery and validation 著者: Najeeha T. Iqbal, Sana Syed, Kamran Sadiq, Marium N. Khan, Junaid Iqbal, Jennie Z. Ma, Fayaz Umrani, Sheraz Ahmed, Elizabeth A. Maier, Lee A. Denson, Yael Haberman, Monica M. McNeal, Kenneth D. R. Setchell, Xueheng Zhao, Shahida Qureshi, Lanlan Shen, Christopher A. Moskaluk, Ta-Chiang Liu, Omer Yilmaz, Donald E. Brown, Michael J. Barratt, Vanderlene L. Kung, Jeffrey I. Gordon, Sean R. Moore, S. Asad Ali

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Machine-learning-based integrative –‘omics analyses reveal immunologic and metabolic dysregulation in environmental enteric dysfunction 著者: Fatima Zulqarnain, Xueheng Zhao, Kenneth D.R. Setchell, Yash Sharma, Phillip Fernandes, Sanjana Srivastava, Aman Shrivastava, Lubaina Ehsan, Varun Jain, Shyam Raghavan, Christopher Moskaluk, Yael Haberman, Lee A. Denson, Khyati Mehta, Najeeha T. Iqbal, Najeeb Rahman, Kamran Sadiq, Zubair Ahmad, Romana Idress, Junaid Iqbal, Sheraz Ahmed, Aneeta Hotwani, Fayyaz Umrani, Beatrice Amadi, Paul Kelly, Donald E. Brown, Sean R. Moore, Syed Asad Ali, Sana Syed

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