Results 61 to 70 of about 9,009 (166)

Upregulation of sphingomyelin and ABCA8 in response to TDP‐43 pathology in amyotrophic lateral sclerosis brain

Brain Pathology, EarlyView.
Increases in sphingomyelin in response to TDP‐43 pathology in the disease‐affected motor cortex of amyotrophic lateral sclerosis (ALS) brain. Abstract Amyotrophic lateral sclerosis (ALS) is a rapidly progressing neurodegenerative disease characterized by the degeneration of motor neurons and the presence of TAR DNA‐binding protein 43 (TDP‐43 ...
Finula I. Isik, Russell Pickford, Nicolas Dzamko, Kerry‐Anne Rye, Woojin Scott Kim   +4 more
wiley   +1 more source

Isolation of Glucosylceramides from Yeast (Hansenula ciferri)

Journal of Biological Chemistry, 1971
Abstract A glucocerebroside was isolated from the yeast Hansenula ciferri. The cerebroside contained only a single sugar, d-glucose, and several species of sphingosine derivatives and fatty acids. Phytosphingosine was detected only in trace amounts in some preparations, and was absent in others, in spite of the fact that the yeast is known to produce ...
B, Kaufman, S, Basu, S, Roseman
openaire   +2 more sources

New aminocyclitols as modulators of glucosylceramide metabolism [PDF]

Org. Biomol. Chem., 2005
A series of 13 aminocyclitol derivatives belonging to two different families is described. Their configuration is governed by the regio- and stereocontrolled epoxide opening of a suitably protected conduritol-B epoxide. Studies on several glycosyl processing enzymes indicate that some of them are good inhibitors of glucosylceramide hydrolase.
Egido-Gabás, Meritxell, Serrano, Pedro, Casas, Josefina, Llebaria, Amadeu, Delgado Cirilo, Antonio   +4 more
openaire   +3 more sources

Ambroxol Hydrochloride Improves Motor Functions and Extends Survival in a Mouse Model of Familial Amyotrophic Lateral Sclerosis

Frontiers in Pharmacology, 2019
Amyotrophic lateral sclerosis (ALS) is a multifactorial and fatal neurodegenerative disease. Growing evidence connects sphingolipid metabolism to the pathophysiology of ALS.
Alexandra Bouscary, Alexandra Bouscary, Cyril Quessada, Cyril Quessada, Althéa Mosbach, Althéa Mosbach, Noëlle Callizot, Michael Spedding, Jean-Philippe Loeffler, Jean-Philippe Loeffler, Alexandre Henriques, Alexandre Henriques, Alexandre Henriques   +12 more
doaj   +1 more source

Iminosugar-based inhibitors of glucosylceramide synthase increase brain glycosphingolipids and survival in a mouse model of Sandhoff disease. [PDF]

PLoS ONE, 2011
The neuropathic glycosphingolipidoses are a subgroup of lysosomal storage disorders for which there are no effective therapies. A potential approach is substrate reduction therapy using inhibitors of glucosylceramide synthase (GCS) to decrease the ...
Karen M Ashe, Dinesh Bangari, Lingyun Li, Mario A Cabrera-Salazar, Scott D Bercury, Jennifer B Nietupski, Christopher G F Cooper, Johannes M F G Aerts, Edward R Lee, Diane P Copeland, Seng H Cheng, Ronald K Scheule, John Marshall   +12 more
doaj   +1 more source

Investigation into the delivery and evaluation of a unique Avena sativa (Oat) Lipid Extract on skin barrier using LC‒MS/MS, Raman spectroscopy, immunostaining analysis and skin efficacy

International Journal of Cosmetic Science, EarlyView.
Abstract Objectives Oat lipids are rich in ceramides. In this investigation, we analysed the delivery of a specific Oat Lipid Extract derived from Avena sativa, comprising ‘skin identical’ ratios of fatty acids, ceramides and cholesterol/sterol. This investigation is the first reported investigation of oat lipids utilizing Raman spectroscopy for the ...
E. Gombert, C. Dewis
wiley   +1 more source

Glucosylceramide synthase inhibition enhances vincristine-induced cytotoxicity [PDF]

International Journal of Cancer, 2001
As a strategy to enhance tumor cell sensitivity to vincristine, we tested whether modulation of sphingolipid metabolism would alter vincristine cytotoxicity since this is linked to accumulation of the intermediate metabolite, ceramide. We blocked ceramide metabolism in a series of variably vincristine-resistant cell lines derived from CCRF-CEM leukemia
R S, Olshefski, S, Ladisch
openaire   +2 more sources

Plant-Unique cis/trans Isomerism of Long-Chain Base Unsaturation is Selectively Required for Aluminum Tolerance Resulting from Glucosylceramide-Dependent Plasma Membrane Fluidity

Plants, 2019
Cis/trans isomerism of the Δ8 unsaturation of long-chain base (LCB) is found only in plant sphingolipids. This unique geometry is generated by sphingolipid LCB Δ8 desaturase SLD which produces both isomers at various ratios, resulting in ...
Masaya Sato, Minoru Nagano, Song Jin, Atsuko Miyagi, Masatoshi Yamaguchi, Maki Kawai-Yamada, Toshiki Ishikawa   +6 more
doaj   +1 more source

Skin Barrier Biomarkers in Patch‐Induced and Clinical Allergic and Irritant Contact Dermatitis

Contact Dermatitis, Volume 94, Issue 4, Page 317-326, April 2026.
Both patch‐induced and clinical ICD and ACD showed consistent alterations in skin barrier‐associated biomarkers, indicative of skin barrier impairment. Among the tested allergens, chromium elicited more pronounced biomarker responses compared to nickel and methylisothiazolinone.
Sanja Kezic, Florentine de Boer, Nariman K. A. Metwally, Karen Ghauharali‐van der Vlugt, Femke S. Beers‐Stet, Wouter Ouwerkerk, Ivone Jakasa, Thomas Rustemeyer, Henk F. van der Molen   +8 more
wiley   +1 more source

Glucosylceramide Synthase Is Involved in Development of Invariant Natural Killer T Cells

Frontiers in Immunology, 2017
Invariant natural killer T (iNKT) cells represent a unique population of CD1d-restricted T lymphocytes expressing an invariant T cell receptor encoded by Vα14-Jα18 and Vα24-Jα18 gene segments in mice and humans, respectively.
Zoran V. Popovic, Zoran V. Popovic, Mariona Rabionet, Richard Jennemann, Damir Krunic, Roger Sandhoff, Hermann-Josef Gröne, Stefan Porubsky   +7 more
doaj   +1 more source