Results 61 to 70 of about 5,986,972 (170)
The clinical spectrum of SMA‐PME and in vitro normalization of its cellular ceramide profile
Annals of Clinical and Translational Neurology, 2022 Objective The objectives of this study were to define the clinical and biochemical spectrum of spinal muscular atrophy with progressive myoclonic epilepsy (SMA‐PME) and to determine if aberrant cellular ceramide accumulation could be normalized by enzyme
Michelle M. Lee +16 more
doaj +1 more source
Nature Communications, 2020 Macrophages are critical in limiting replication of herpes simplex virus type 1 (HSV-1). Here the authors show how acid ceramidase and its enzymatic product sphingosine enable multivesicular bodies to function as an anti-viral mechanism.
Judith Lang +30 more
doaj +1 more source
Activation of Ceramidase and Ceramide Kinase by Vanadate via a Tyrosine Kinase–Mediated Pathway
Journal of Pharmacological Sciences, 2010 .: Ceramide, a key molecule in the metabolism of sphingolipids, is converted by ceramidase to sphingosine, and phosphorylated by ceramide kinase to form ceramide-1-phosphate (C1P). In this study, we improved on a method of thin-layer chromatography using
Eiko Tada +7 more
doaj +1 more source
Sphingoid long chain bases prevent lung infection by Pseudomonas aeruginosa
EMBO Molecular Medicine, 2014 Cystic fibrosis patients and patients with chronic obstructive pulmonary disease, trauma, burn wound, or patients requiring ventilation are susceptible to severe pulmonary infection by Pseudomonas aeruginosa.
Yael Pewzner‐Jung +14 more
doaj +1 more source
Redox Biology, 2021 Niemann-Pick type C (NPC) disease, a lysosomal storage disorder caused by defective NPC1/NPC2 function, results in the accumulation of cholesterol and glycosphingolipids in lysosomes of affected organs, such as liver and brain.
S. Torres +11 more
semanticscholar +1 more source
Development of an acid ceramidase activity-based probe
Chemical Communications, 2015 Acid ceramidase is responsible for the ultimate step in the catabolism of (glyco)sphingolipids by hydrolysis of ceramide into sphingosine and free fatty acid.
Ouairy, C.M.J. +9 more
openaire +3 more sources
Angewandte Chemie International Edition, EarlyView.A 310‐helix‐mediated conformational switch promotes a front‐face SNi‐like catalysis by human A4GALT. Mechanism‐guided design identifies AdaGalCer as a selective modulator of globotriaosylceramide (Gb3) biosynthesis, opening a clear route toward new Fabry disease therapeutics.
Nicky de Koster +13 more
wiley +1 more source
Data in Brief, 2018 Ceramide pathway is a key regulator in cell proliferation and apoptosis and oxidative stress up-regulate ceramides. Acid ceramidase (ACDase), neutral sphingomyelinase (NSMase) and glucosylceramide synthase (GCS) are critical enzymatic systems in ceramide
Mahdi Mashhadi Akbar Boojar +3 more
doaj +1 more source
Acid ceramidase confers radioresistance to glioblastoma cells
Oncology Reports, 2017 Glioblastoma multiforme (GBM) is the most common primary, intracranial malignancy of the central nervous system. The standard treatment protocol, which involves surgical resection, and concurrent radiation with adjuvant temozolomide (TMZ), still imparts a grim prognosis.
Doan, Ninh B. +9 more
openaire +3 more sources
Movement Disorders, EarlyView.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 +21 more
wiley +1 more source