Search Results - "Park, HyeJung"

Glucosylceramide synthase inhibition alleviates aberrations in synucleinopathy models by Sardi, S. Pablo, Viel, Catherine, Clarke, Jennifer, Treleaven, Christopher M., Richards, Amy M., Park, Hyejung, Olszewski, Maureen A., Dodge, James C., Marshall, John, Makino, Elina, Wang, Bing, Sidman, Richard L., Cheng, Seng H., Shihabuddin, Lamya S.

“…Mutations in the glucocerebrosidase gene (GBA) confer a heightened risk of developing Parkinson’s disease (PD) and other synucleinopathies, resulting in a…”
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CNS-accessible Inhibitor of Glucosylceramide Synthase for Substrate Reduction Therapy of Neuronopathic Gaucher Disease by Marshall, John, Sun, Ying, Bangari, Dinesh S, Budman, Eva, Park, Hyejung, Nietupski, Jennifer B, Allaire, Amy, Cromwell, Mary A, Wang, Bing, Grabowski, Gregory A, Leonard, John P, Cheng, Seng H

“…Gaucher disease (GD) is caused by a deficiency of glucocerebrosidase and the consequent lysosomal accumulation of unmetabolized glycolipid substrates…”
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Substrate reduction therapy using Genz‐667161 reduces levels of pathogenic components in a mouse model of neuronopathic forms of Gaucher disease by Blumenreich, Shani, Yaacobi, Chen, Vardi, Ayelet, Barav, Or B., Vitner, Einat B., Park, Hyejung, Wang, Bing, Cheng, Seng H., Sardi, Sergio P., Futerman, Anthony H.

“…Most lysosomal storage diseases (LSDs) have a significant neurological component, including types 2 and 3 Gaucher disease (neuronal forms of Gaucher disease;…”
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Quantitative analysis of sphingolipids for lipidomics using triple quadrupole and quadrupole linear ion trap mass spectrometers[S] by Shaner, Rebecca L., Allegood, Jeremy C., Park, Hyejung, Wang, Elaine, Kelly, Samuel, Haynes, Christopher A., Sullards, M. Cameron, Merrill, Alfred H.

“…Sphingolipids are a highly diverse category of bioactive compounds. This article describes methods that have been validated for the extraction, liquid…”
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Direct analysis of sialylated or sulfated glycosphingolipids and other polar and neutral lipids using TLC-MS interfaces[S] by Park, Hyejung, Zhou, Ying, Costello, Catherine E.

“…Gangliosides and sulfatides (STs) are acidic glycosphingolipids (GSLs) that have one or more sialic acids or sulfate substituents, in addition to neutral…”
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Sphingolipidomics: Methods for the comprehensive analysis of sphingolipids by Haynes, Christopher A., Allegood, Jeremy C., Park, Hyejung, Sullards, M. Cameron

“…Sphingolipids comprise a highly diverse and complex class of molecules that serve as both structural components of cellular membranes and signaling molecules…”
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Glucosylceramide synthase inhibition protects against cardiac hypertrophy in chronic kidney disease by Baccam, Gabriel C., Xie, Jian, Jin, Xin, Park, Hyejung, Wang, Bing, Husson, Hervé, Ibraghimov-Beskrovnaya, Oxana, Huang, Chou-Long

“…A significant population of patients with chronic kidney disease (CKD) develops cardiac hypertrophy, which can lead to heart failure and sudden cardiac death…”
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Preclinical pharmacology of glucosylceramide synthase inhibitor venglustat in a GBA-related synucleinopathy model by Viel, Catherine, Clarke, Jennifer, Kayatekin, Can, Richards, Amy M., Chiang, Ming Sum R., Park, Hyejung, Wang, Bing, Shihabuddin, Lamya S., Sardi, S. Pablo

“…Mutations in GBA , the gene encoding the lysosomal enzyme glucocerebrosidase (GCase), represent the greatest genetic risk factor for developing…”
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Human iPSC-derived astrocytes generated from donors with globoid cell leukodystrophy display phenotypes associated with disease by Lieberman, Richard, Cortes, Leslie K, Gao, Grace, Park, Hyejung, Wang, Bing, Jones, Patrick L, Hunter, R. Bridge, Leonard, John P, Barker, Robert H

“…Globoid cell leukodystrophy (Krabbe disease) is a fatal neurodegenerative, demyelinating disease caused by dysfunctional activity of galactosylceramidase…”
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A novel brain-penetrant oral UGT8 inhibitor decreases in vivo galactosphingolipid biosynthesis in murine Krabbe disease by Zaccariotto, Eva, Cachón-González, María Begoña, Wang, Bing, Lim, Sungtaek, Hirth, Bradford, Park, Hyejung, Fezoui, Malika, Sardi, S.Pablo, Mason, Paul, Barker, Robert H., Cox, Timothy M.

“…Krabbe disease is a rare, inherited neurodegenerative disease due to impaired lysosomal β-galactosylceramidase (GALC) activity and formation of neurotoxic…”
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Lipid Sorting by Ceramide Structure from Plasma Membrane to ER for the Cholera Toxin Receptor Ganglioside GM1 by Chinnapen, Daniel J.-F., Hsieh, Wan-Ting, te Welscher, Yvonne M., Saslowsky, David E., Kaoutzani, Lydia, Brandsma, Eelke, D'Auria, Ludovic, Park, Hyejung, Wagner, Jessica S., Drake, Kimberly R., Kang, Minchul, Benjamin, Thomas, Ullman, M. David, Costello, Catherine E., Kenworthy, Anne K., Baumgart, Tobias, Massol, Ramiro H., Lencer, Wayne I.

“…The glycosphingolipid GM1 binds cholera toxin (CT) on host cells and carries it retrograde from the plasma membrane (PM) through endosomes, the trans-Golgi…”
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Association between the presence of delirium during intensive care unit admission and cognitive impairment or psychiatric problems: the Korean ICU National Data Study by Ko, Ryoung-Eun, Kang, Danbee, Park, Hyejung, Cho, Juhee, Suh, Gee Young, Chung, Chi Ryang

“…Delirium in the intensive care unit (ICU) may be a preventable risk factor for cognitive impairment or psychiatric problems. We aimed to evaluate the…”
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Sphingosine 1-Phosphate (S1P) Lyase Deficiency Increases Sphingolipid Formation via Recycling at the Expense of de Novo Biosynthesis in Neurons by Hagen-Euteneuer, Nadine, Lütjohann, Dieter, Park, Hyejung, Merrill, Alfred H., van Echten-Deckert, Gerhild

“…Sphingosine 1-phosphate lyase (S1P lyase) irreversibly cleaves sphingosine 1-phosphate (S1P) in the final step of sphingolipid catabolism. As sphingoid bases…”
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Sphingolipidomics: a valuable tool for understanding the roles of sphingolipids in biology and disease by Merrill, Alfred H., Stokes, Todd H., Momin, Amin, Park, Hyejung, Portz, Brent J., Kelly, Samuel, Wang, Elaine, Sullards, M. Cameron, Wang, May Dongmei

“…The sphingolipidome is the portion of the lipidome that encompasses all sphingoid bases and their derivatives. Whereas the most studied sphingoid base is…”
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Subcellular Origin of Sphingosine 1-Phosphate Is Essential for Its Toxic Effect in Lyase-deficient Neurons by Hagen, Nadine, Van Veldhoven, Paul P., Proia, Richard L., Park, Hyejung, Merrill, Alfred H., van Echten-Deckert, Gerhild

“…Cerebellar granule cells from sphingosine 1-phosphate (S1P) lyase-deficient mice were used to study the toxicity of this potent sphingolipid metabolite in…”
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Forecasting the Walking Assistance Rehabilitation Level of Stroke Patients Using Artificial Intelligence by Seo, Kanghyeon, Chung, Bokjin, Panchaseelan, Hamsa Priya, Kim, Taewoo, Park, Hyejung, Oh, Byungmo, Chun, Minho, Won, Sunjae, Kim, Donkyu, Beom, Jaewon, Jeon, Doyoung, Yang, Jihoon

“…Cerebrovascular accidents (CVA) cause a range of impairments in coordination, such as a spectrum of walking impairments ranging from mild gait imbalance to…”
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Glucosylceramide in cerebrospinal fluid of patients with GBA-associated and idiopathic Parkinson’s disease enrolled in PPMI by Huh, Young Eun, Park, Hyejung, Chiang, Ming Sum Ruby, Tuncali, Idil, Liu, Ganqiang, Locascio, Joseph J., Shirvan, Julia, Hutten, Samantha J., Rotunno, Melissa S., Viel, Catherine, Shihabuddin, Lamya S., Wang, Bing, Sardi, Sergio Pablo, Scherzer, Clemens R.

“…Protein-coding variants in the GBA gene modulate susceptibility and progression in ~10% of patients with Parkinson’s disease (PD). GBA encodes the…”
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Relationship between Use of Rehabilitation Resources and ICU Readmission and ER Visits in ICU Survivors: the Korean ICU National Data Study 2008-2015 by Park, Yun Hee, Ko, Ryoung Eun, Kang, Danbee, Park, Jinkyeong, Jeon, Kyeongman, Yang, Jeong Hoon, Park, Chi Min, Cho, Joongbum, Park, Young Sook, Park, Hyejung, Cho, Juhee, Guallar, Eliseo, Suh, Gee Young, Chung, Chi Ryang

“…Despite the increasing importance of rehabilitation for critically ill patients, there is little information regarding how rehabilitation therapy is utilized…”
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The galactocerebrosidase enzyme contributes to the maintenance of a functional hematopoietic stem cell niche by Visigalli, Ilaria, Ungari, Silvia, Martino, Sabata, Park, Hyejung, Cesani, Martina, Gentner, Bernhard, Sergi Sergi, Lucia, Orlacchio, Aldo, Naldini, Luigi, Biffi, Alessandra

“…The balance between survival and death in many cell types is regulated by small changes in the intracellular content of bioactive sphingolipids. Enzymes that…”
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Potential role of acid ceramidase in conversion of cytostatic to cytotoxic end-point in pancreatic cancer cells by Morad, Samy A. F., Messner, Maria C., Levin, Jonathan C., Abdelmageed, Noha, Park, Hyejung, Merrill, Alfred H., Cabot, Myles C.

“…Purpose Acid ceramidase (AC) occupies an important place in the control of cancer cell proliferation. We tested the influence of AC inhibition on the effects…”
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