Results 21 to 30 of about 31,833 (220)

Porcine intestinal glycosphingolipids recognized by F6-fimbriated enterotoxigenic Escherichia coli [PDF]

, 2014
One important virulence factor of enterotoxigenic Escherichia coli is their ability to adhere via fimbrial adhesins to specific receptors located on the intestinal mucosa. Here, the potential glycosphingolipid receptors of enterotoxigenic F6-fimbriated E.
Benktander, John, Coddens, Annelies, Cox, Eric, Johansson, Miralda Madar, Teneberg, Susann   +4 more
core   +2 more sources

An Inducible Mouse Model of Late Onset Tay–Sachs Disease

Neurobiology of Disease, 2002
Mouse models of the GM2 gangliosidoses, Tay–Sachs and Sandhoff disease, are null for the hexosaminidase α and β subunits respectively. The Sandhoff (Hexb−/−) mouse has severe neurological disease and mimics the human infantile onset variant. However, the
Mylvaganam Jeyakumar, David Smith, Elena Eliott-Smith, Mario Cortina-Borja, Gabriele Reinkensmeier, Terry D. Butters, Thorsten Lemm, Konrad Sandhoff, V.Hugh Perry, Raymond A. Dwek, Frances M. Platt   +10 more
doaj   +1 more source

The role of neuraminidase 1 (NEU1) in cytokine release by primary mouse mesangial cells and disease outcomes in murine lupus nephritis

Autoimmunity, 2021
The importance of altered glycosphingolipid (GSL) metabolism is increasingly gaining attention as a characteristic of multiple chronic kidney diseases. Previously, we reported elevated levels of GSLs and neuraminidase (NEU) enzyme activity/expression in ...
Jessalyn Rodgers, Kamala Sundararaj, Evelyn Bruner, Bethany Wolf, Tamara K. Nowling   +4 more
doaj   +1 more source

Glycosphingolipid Levels in Urine Extracellular Vesicles Enhance Prediction of Therapeutic Response in Lupus Nephritis

Metabolites, 2022
The development of nephritis increases the risk of morbidity and mortality in systemic lupus erythematosus (SLE) patients. While standard induction therapies, such as mycophenolate mofetil (MMF) induce clinical remission (i.e., complete response) in ...
Brian Troyer, Jessalyn Rodgers, Bethany J. Wolf, James C. Oates, Richard R. Drake, Tamara K. Nowling   +5 more
doaj   +1 more source

Cytosolic Glucosylceramide regulates endolysosomal function in Niemann-Pick type C disease [PDF]

, 2019
A new paradigm for Niemann-Pick C disease is presented where lysosomal storage leads to a deficit in cytoplasmic glucosylceramide (GlcCer) where it performs important functions.
Bhardwaj, Meenakshi, Haberkant, Per, Schmid, Ralf, Sillence, Daniel J., Tongue, Paige, Wheeler, S.   +5 more
core   +1 more source

C-elegans model identifies genetic modifiers of alpha-synuclein inclusion formation during aging [PDF]

, 2008
Inclusions in the brain containing alpha-synuclein are the pathological hallmark of Parkinson's disease, but how these inclusions are formed and how this links to disease is poorly understood.
AA Cooper, AB Singleton, AD Gitler, B Rogina, C Hertzman, CD Link, E Berezikov, E Cuppen, EA Nollen, Ellen A. A. Nollen, GM Halliday, HA Tissenbaum, HG van, HH Liou, HM Beere, JF Morley, Karen L. Thijssen, L Chen, M Kaeberlein, M Kaeberlein, M Nishimura, M Viswanathan, MC Chartier-Harlin, MG Spillantini, MG Spillantini, MH Polymeropoulos, NB Cole, PK Auluck, Rainer Breitling, Robert M. W. Hofstra, Ronald H. A. Plasterk, S Kim, S Miksys, S Roy, S Willingham, Stuart K. Kim, T Kai, TF Outeiro, Tjakko J. van Ham   +38 more
core   +12 more sources

Alterations of Glycosphingolipid Glycans and Chondrogenic Markers during Differentiation of Human Induced Pluripotent Stem Cells into Chondrocytes

Biomolecules, 2020
Due to the limited intrinsic healing potential of cartilage, injury to this tissue may lead to osteoarthritis. Human induced pluripotent stem cells (iPSCs), which can be differentiated into chondrocytes, are a promising source of cells for cartilage ...
Liang Xu, Hisatoshi Hanamatsu, Kentaro Homan, Tomohiro Onodera, Takuji Miyazaki, Jun-ichi Furukawa, Kazutoshi Hontani, Yuan Tian, Rikiya Baba, Norimasa Iwasaki   +9 more
doaj   +1 more source

Characterization of Cell Glycocalyx with Mass Spectrometry Methods. [PDF]

, 2019
The cell membrane plays an important role in protecting the cell from its extracellular environment. As such, extensive work has been devoted to studying its structure and function.
Lebrilla, Carlito B, Li, Qiongyu, Wong, Maurice, Xie, Yixuan   +3 more
core   +2 more sources

Glucosylceramide modulates membrane traffic along the endocytic pathway

Journal of Lipid Research, 2002
Glycosphingolipids are endocytosed and targeted to the Golgi apparatus, but are mistargeted to lysosomes in numerous sphingolipidoses. Substrate reduction therapy utilizes imino sugars to inhibit glucosylceramide synthase and potentially abrogate the ...
Dan J. Sillence, Vishwajeet Puri, David L. Marks, Terry D. Butters, Raymond A. Dwek, Richard E. Pagano, Frances M. Platt   +6 more
doaj   +1 more source

A novel glucosylceramide synthase inhibitor attenuates alpha synuclein pathology and lysosomal dysfunction in preclinical models of synucleinopathy

Neurobiology of Disease, 2021
Mutations in the lysosomal enzyme glucocerebrosidase (GCase, GBA1 gene) are the most common genetic risk factor for developing Parkinson's disease (PD).
Mali Cosden, Sarah Jinn, Lihang Yao, Cheryl A. Gretzula, Monika Kandebo, Dawn Toolan, Nathan G. Hatcher, Lei Ma, Wei Lemaire, Gregory C. Adam, Christine Burlein, Christina Minnick, Rose Flick, Marla L. Watt, James Mulhearn, Mark Fraley, Robert E. Drolet, Jacob N. Marcus, Sean M. Smith   +18 more
doaj   +1 more source