Results 61 to 70 of about 2,629 (119)

Glucosylceramide Critically Contributes to the Host Defense of Cystic Fibrosis Lungs

, 2017
Background: Cystic fibrosis (CF) is the most common autosomal-recessive disorder in western countries. Previous studies have demonstrated an important role of sphingolipids in the pathophysiology of cystic fibrosis.
Barbara Kovacic, Carolin Sehl, Markus Kamler, Katrin Anne Becker, Barbara Wilker, Erich Gulbins   +5 more
core   +1 more source

Hydroxylated (OH) galactosylceramide and glucosylceramide species in iPSC-derived astrocytes.

, 2022
(A) Hydroxylated galactosylceramide was higher in Krabbe astrocytes compared to controls. Examination of the species of OH-galactosylceramide expressed in our cell cultured revealed this increase was driven by C16 OH-galactosylceramide.
Hyejung Park (1322841), Patrick L. Jones (13194701), R. Bridge Hunter (13194704), Richard Lieberman (13194698), Leslie K. Cortes (7377488), Bing Wang (126120), Robert H. Barker Jr. (13194707), John P. Leonard (8755311), Grace Gao (7066793)   +8 more
core   +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

Rare‐Variant Burden across Lysosomal Genes Implicates Sialylation and Ganglioside Metabolism in Parkinson's Disease

Movement Disorders, Volume 41, Issue 9, Page 2349-2356, September 2026.
Abstract Background Lysosomal dysfunction is central to Parkinson's disease (PD) pathogenesis, with GBA1 representing the strongest established genetic risk factor. Numerous other genes involved in lysosomal sphingolipid, glycosphingolipid, and ceramide metabolism have been proposed as contributors to PD, highlighting the need for genetic analyses ...
Konstantin Senkevich, Sitki Cem Parlar, Cloe Chantereault, Lang Liu, Eric Yu, Uladzislau Rudakou, Jamil Ahmad, Jennifer A. Ruskey, Farnaz Asayesh, Dan Spiegelman, Cheryl Waters, Oury Monchi, Yves Dauvilliers, Nicolas Dupré, Lior Greenbaum, Sharon Hassin‐Baer, Irina Miliukhina, Alla Timofeeva, Anton Emelyanov, Sofya Pchelina, Roy N. Alcalay, Ziv Gan‐Or   +21 more
wiley   +1 more source

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

Functional Specialization of Clathrin‐Independent Endocytic Routes: Mechanisms and Cellular Roles

Traffic, Volume 27, Issue 3, September 2026.
Distinct clathrin‐independent endocytic (CIE) routes support specialized cellular functions across different contexts. Created with Biorender.com. ABSTRACT Clathrin‐independent endocytosis (CIE) comprises a diverse repertoire of internalization mechanisms that operate in parallel with clathrin‐mediated endocytosis.
Andrea Francesco Benvenuto, Amir Fardin, Matteo Balestra, Pier Paolo Di Fiore, Chiara Tordonato, Sara Sigismund   +5 more
wiley   +1 more source

Can P-glycoprotein influence the bioavailability of iminosugar-based glucosylceramide synthase inhibitors?

, 2006
Recently developed glucosylceramide synthase inhibitors with enhanced hydrophobicity display increased bioavailability in the central nervous system (CNS). Have these improvements come at a potential risk given that the improved glucosylceramide synthase
Modok, S, Norris-Cervetto, E, Callaghan, R, Dwek, RA, Martin, C, Butters, TD   +5 more
core   +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

Obesity Modifies Sphingolipid Signatures in Childhood Asthma

Clinical &Experimental Allergy, EarlyView.
Harshita Shailesh, Alaa Abdelhamid, Ahmed Arabi, Yaman Al‐Haneedi, Shana Jacob, Giridhara Rathnaiah Babu, Stefan Worgall, Ibrahim Janahi   +7 more
wiley   +1 more source

A Systematic Review on Disease‐Modifying Therapies in Parkinsonian Disorders

Clinical Pharmacology &Therapeutics, Volume 120, Issue 2, Page 357-374, August 2026.
Parkinsonian disorders, including Parkinson's disease, Lewy body dementia, multiple system atrophy, and progressive supranuclear palsy, are progressive neurodegenerative conditions with no treatment options to slow disease progression. This systematic review provides an overview of evidence of disease‐modifying therapies that have been evaluated in ...
Pepijn P.N.M. Eijsvogel, Lars M.G. Smits, Philip H.C. Kremer, Geert Jan Groeneveld   +3 more
wiley   +1 more source