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Xun-Tian Jiang · SciRah

Researcher profile · Semantic Scholar

Xun-Tian Jiang

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Xun-Tian Jiang — researcher profile on SciRah

h-index: 30
Works: 90
Citations: 3,693

Most-cited papers

Lysosomal Cholesterol Activates mTORC1 via an SLC38A9-Niemann Pick C1 Signaling Complex
Brian M. Castellano, Ashley M. Thelen, Ofer Moldavski, McKenna Feltes +11 more
2017Science506 citations Open access
Semantic Scholar
ER-lysosome contacts enable cholesterol sensing by mTORC1 and drive aberrant growth signaling in Niemann-Pick type C
Chun-Yan Lim, Oliver B. Davis, Hijai R. Shin, Justin Zhang +6 more
2019Nature Cell Biology280 citations Open access
Semantic Scholar
Intrathecal 2-hydroxypropyl-β-cyclodextrin decreases neurological disease progression in Niemann-Pick Disease, type C1: an ad-hoc analysis of a non-randomized, open-label, phase 1/2 trial
D. Ory, E. Ottinger, N. Farhat, K. King +23 more
2017The Lancet268 citations Open access
Semantic Scholar
A sensitive and specific LC-MS/MS method for rapid diagnosis of Niemann-Pick C1 disease from human plasma[S]
Xun-Tian Jiang, Rohini Sidhu, F. Porter, N. Yanjanin +9 more
2011Journal of Lipid Research175 citations Open access
Semantic Scholar
Ceramide Remodeling and Risk of Cardiovascular Events and Mortality
L. Peterson, V. Xanthakis, Meredith S. Duncan, S. Gross +11 more
2018Journal of the American Heart Association : Cardiovascular and Cerebrovascular Disease157 citations Open access
Semantic Scholar
Alterations in lipid homeostasis of mouse dorsal root ganglia induced by apolipoprotein E deficiency: a shotgun lipidomics study
Hua Cheng, Xun-Tian Jiang, Xian-Lin Han
2006Journal of Neurochemistry108 citations Open access
Semantic Scholar
AAV gene therapy for Tay-Sachs disease
T. Flotte, O. Cataltepe, A. Puri, A. Batista +26 more
2022Nature Medicine100 citations Open access
Semantic Scholar
Development of a Bile Acid-Based Newborn Screen for Niemann-Pick C Disease
Xun-Tian Jiang, Rohini Sidhu, Laurel Mydock-McGrane, F. Hsu +16 more
2016Science Translational Medicine96 citations Open access
Semantic Scholar
Alkaline methanolysis of lipid extracts extends shotgun lipidomics analyses to the low-abundance regime of cellular sphingolipids.
Xun-Tian Jiang, Hua Cheng, Kui Yang, R. Gross +1 more
2007Analytical Biochemistry89 citations Open access
Semantic Scholar
Shotgun lipidomics reveals the temporally dependent, highly diversified cardiolipin profile in the mammalian brain: temporally coordinated postnatal diversification of cardiolipin molecular species with neuronal remodeling.
Hua Cheng, D. J. Mancuso, Xun-Tian Jiang, Shaoping Guan +5 more
2008Biochemistry88 citations Open access
Semantic Scholar
Mechanism-Based Combination Treatment Dramatically Increases Therapeutic Efficacy in Murine Globoid Cell Leukodystrophy
Jacqueline A. Hawkins-Salsbury, L. Shea, Xun-Tian Jiang, D. Hunter +7 more
2015Journal of Neuroscience87 citations Open access
Semantic Scholar
Genetic ablation of acid ceramidase in Krabbe disease confirms the psychosine hypothesis and identifies a new therapeutic target
Yedda Li, Yue Xu, B. Benitez, Murtaza S. Nagree +15 more
2019Proceedings of the National Academy of Sciences of the United States of America86 citations Open access
Semantic Scholar
Relative acidic compartment volume as a lysosomal storage disorder-associated biomarker.
Danielle Taylor Te Vruchte, A. Speak, Kerri-Lee Wallom, Nada Al Eisa +32 more
2014Journal of Clinical Investigation86 citations Open access
Semantic Scholar
Characterization of oxysterols by electrospray ionization tandem mass spectrometry after one-step derivatization with dimethylglycine.
Xun-Tian Jiang, D. Ory, Xian-Lin Han
2007Rapid Communications in Mass Spectrometry86 citations Open access
Semantic Scholar
Intrathecal 2-Hydroxypropyl-Beta-Cyclodextrin in a Single Patient with Niemann-Pick C1
T. Maarup, Agnes H. Chen, F. Porter, N. Farhat +4 more
2015Molecular Genetics and Metabolism79 citations Open access
Semantic Scholar
Eggs early in complementary feeding increase choline pathway biomarkers and DHA: a randomized controlled trial in Ecuador
L. Iannotti, C. Lutter, W. Waters, Carlos Andres Gallegos Riofrío +16 more
2017American Journal of Clinical Nutrition78 citations Open access
Semantic Scholar
Psychosine, the cytotoxic sphingolipid that accumulates in globoid cell leukodystrophy, alters membrane architecture
Jacqueline A. Hawkins-Salsbury, Archana R. Parameswar, Xun-Tian Jiang, P. Schlesinger +4 more
2013Journal of Lipid Research70 citations Open access
Semantic Scholar
A review of lipidomic technologies applicable to sphingolipidomics and their relevant applications
Xian-Lin Han, Xun-Tian Jiang
2009European Journal of Lipid Science and Technology70 citations Open access
Semantic Scholar
A validated LC-MS/MS assay for quantification of 24(S)-hydroxycholesterol in plasma and cerebrospinal fluid
Rohini Sidhu, Hui Jiang, N. Farhat, Nuria Carrillo-Carrasco +6 more
2015Journal of Lipid Research67 citations Open access
Semantic Scholar
Characterization and direct quantitation of sphingoid base-1-phosphates from lipid extracts: a shotgun lipidomics approach Published, JLR Papers in Press, May 8, 2006.
Xun-Tian Jiang, Xian-Lin Han
2006Journal of Lipid Research59 citations Open access
Semantic Scholar
Krabbe disease successfully treated via monotherapy of intrathecal gene therapy.
Allison M. Bradbury, Jessica H. Bagel, Duc V. M. Nguyen, Erik A. Lykken +15 more
2020Journal of Clinical Investigation51 citations Open access
Semantic Scholar
A novel gene editing system to treat both Tay-Sachs and Sandhoff diseases
Li Ou, M. Przybilla, A. Tabaran, P. Overn +6 more
2020Gene Therapy48 citations Open access
Semantic Scholar
Cholesterol homeostatic responses provide biomarkers for monitoring treatment for the neurodegenerative disease Niemann-Pick C1 (NPC1).
Brett A. Tortelli, H. Fujiwara, J. Bagel, Jessie Zhang +11 more
2014Human Molecular Genetics48 citations Open access
Semantic Scholar
Bone marrow transplantation increases efficacy of central nervous system-directed enzyme replacement therapy in the murine model of globoid cell leukodystrophy
Elizabeth Y. Qin, Jacqueline A. Hawkins-Salsbury, Xun-Tian Jiang, Adarsh Reddy +3 more
2012Molecular Genetics and Metabolism46 citations Open access
Semantic Scholar
Enhanced Efficacy and Increased Long-Term Toxicity of CNS-Directed, AAV-Based Combination Therapy for Krabbe disease.
Yedda Li, Christopher A. Miller, L. Shea, Xun-Tian Jiang +9 more
2020Molecular Therapy44 citations Open access
Semantic Scholar
Neural stem cells for disease modeling and evaluation of therapeutics for Tay-Sachs disease
Mylinh Vu, Rong Li, Amanda Baskfield, Billy Lu +14 more
2018Orphanet Journal of Rare Diseases42 citations Open access
Semantic Scholar
N-acyl-O-phosphocholineserines: structures of a novel class of lipids that are biomarkers for Niemann-Pick C1 disease
Rohini Sidhu, Yawo A. Mondjinou, Mingxing Qian, Hao-Wei Song +12 more
2019Journal of Lipid Research39 citations Open access
Semantic Scholar
Development and validation of LC-MS/MS method for determination of very long acyl chain (C22:0 and C24:0) ceramides in human plasma
Hui Jiang, F. Hsu, Marsha S. Farmer, L. Peterson +3 more
2013Analytical and Bioanalytical Chemistry38 citations Open access
Semantic Scholar
Endosomes and lysosomes play distinct roles in sulfatide-induced neuroblastoma apoptosis: potential mechanisms contributing to abnormal sulfatide metabolism in related neuronal diseases.
You-Chun Zeng, Hua Cheng, Xun-Tian Jiang, Xian-Lin Han
2008Biochemical Journal38 citations
Semantic Scholar
Loss of SNORA73 reprograms cellular metabolism and protects against steatohepatitis
A. Sletten, J. Davidson, Büsra Yagabasan, Samantha Moores +10 more
2021Nature Communications34 citations Open access
Semantic Scholar
Application of N-Palmitoyl-O-Phosphocholineserine for Diagnosis and Assessment of Response to Treatment in Niemann-Pick Type C disease
Rohini Sidhu, P. Kell, D. Dietzen, N. Farhat +14 more
2020Molecular Genetics and Metabolism29 citations Open access
Semantic Scholar
Pazopanib plus cetuximab in recurrent or metastatic head and neck squamous cell carcinoma: an open-label, phase 1b and expansion study
D. Adkins, P. Mehan, J. Ley, M. Siegel +6 more
2018The Lancet Oncology29 citations Open access
Semantic Scholar
Diagnosis of Niemann-Pick C1 by Measurement of Bile Acid Biomarkers in Archived Newborn Dried Blood Spots
Xun-Tian Jiang, Rohini Sidhu, Joseph J. Orsini, N. Farhat +4 more
2018Molecular Genetics and Metabolism28 citations Open access
Semantic Scholar
Comprehensive behavioral and biochemical outcomes of novel murine models of GM1-gangliosidosis and Morquio syndrome type B.
M. Przybilla, Li Ou, A. Tabaran, Xun-Tian Jiang +5 more
2019Molecular Genetics and Metabolism27 citations Open access
Semantic Scholar
Niemann-Pick disease type C clinical database: cognitive and coordination deficits are early disease indicators
Miriam Stampfer, Susanne Theiss, Y. Amraoui, Xun-Tian Jiang +5 more
2013Orphanet Journal of Rare Diseases26 citations Open access
Semantic Scholar
Advancing Diagnosis and Treatment of Niemann-Pick C disease through Biomarker Discovery
Xun-Tian Jiang, D. Ory
2021Exploration of Neuroprotective Therapy25 citations Open access
Semantic Scholar
Lipidomic Evaluation of Feline Neurologic Disease after AAV Gene Therapy
H. Gray-Edwards, Xun-Tian Jiang, Ashley N. Randle, Amanda R. Taylor +6 more
2017Molecular Therapy: Methods & Clinical Development24 citations Open access
Semantic Scholar
4β-Hydroxycholesterol is a prolipogenic factor that promotes SREBP1c expression and activity through the liver X receptor
Ofer Moldavski, Peter-James H. Zushin, Charles A. Berdan, R. J. van Eijkeren +8 more
2021Journal of Lipid Research23 citations Open access
Semantic Scholar
Genetic Architecture of Circulating Very-Long-Chain (C24:0 and C22:0) Ceramide Concentrations
S. Cresci, Rui-Bo Zhang, Qiong Yang, Meredith S. Duncan +5 more
2020Journal of Lipid and Atherosclerosis23 citations Open access
Semantic Scholar
Simple nutrients bypass the requirement for HLH-30 in coupling lysosomal nutrient sensing to survival
John T. Murphy, Haiyan Liu, Xiu-Cui Ma, A. Shaver +14 more
2019PLoS Biology22 citations Open access
Semantic Scholar
2-hydroxypropyl-β-cyclodextrin is the active component in a triple combination formulation for treatment of Niemann-Pick C1 Disease
J. Davidson, Elizabeth A. Molitor, Samantha Moores, S. Gale +22 more
2019Biochimica et Biophysica Acta - Molecular and Cell Biology of Lipids22 citations Open access
Semantic Scholar
A HILIC-MS/MS Method for Simultaneous Quantification of the Lysosomal Disease Markers Galactosylsphingosine and Glucosylsphingosine in Mouse Serum
Rohini Sidhu, Christina R. Mikulka, H. Fujiwara, M. Sands +3 more
2018Biomedical chromotography22 citations Open access
Semantic Scholar
Clinical, electrophysiological, and biochemical markers of peripheral and central nervous system disease in canine globoid cell leukodystrophy (Krabbe disease)
Allison M. Bradbury, J. Bagel, Xun-Tian Jiang, Gary P. Swain +6 more
2016Journal of Neuroscience Research22 citations Open access
Semantic Scholar
Development and validation of sensitive LC-MS/MS assays for quantification of HP-β-CD in human plasma and CSF
Hui Jiang, Rohini Sidhu, H. Fujiwara, M. De Meulder +11 more
2014Journal of Lipid Research22 citations Open access
Semantic Scholar
A human iPSC-derived inducible neuronal model of Niemann-Pick disease, type C1
Anika V. Prabhu, Insung Kang, Raffaella De Pace, C. Wassif +7 more
2021BMC Biology21 citations Open access
Semantic Scholar
Characterization of long-chain fatty acid as N-(4-aminomethylphenyl) pyridinium derivative by MALDI LIFT-TOF/TOF mass spectrometry
C. Frankfater, Xun-Tian Jiang, F. Hsu
2018Journal of the American Society for Mass Spectrometry21 citations Open access
Semantic Scholar
Sphingomyelin 16:0 is a therapeutic target for neuronal death in acid sphingomyelinase deficiency
Á. Gaudioso, Xun-Tian Jiang, J. Casas, E. Schuchman +1 more
2023Cell Death and Disease20 citations Open access
Semantic Scholar
Substrate reduction therapy for Krabbe disease and metachromatic leukodystrophy using a novel ceramide galactosyltransferase inhibitor
M. Babcock, Christina R. Mikulka, Bing Wang, S. Chandriani +15 more
2021Scientific Reports20 citations Open access
Semantic Scholar
Cerebrospinal fluid and serum glycosphingolipid biomarkers in canine globoid cell leukodystrophy (Krabbe Disease)
C. Corado, J. Pinkstaff, Xun-Tian Jiang, Evelyn M. Galban +8 more
2019Molecular and cellular neurosciences20 citations Open access
Semantic Scholar
Global Biochemical Profiling Identifies β-Hydroxypyruvate as a Potential Mediator of Type 2 Diabetes in Mice and Humans
Sheng Zhang, Song-Yan Wang, M. Puhl, Xun-Tian Jiang +6 more
2014Diabetes20 citations Open access
Semantic Scholar

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