Results 41 to 50 of about 7,301 (235)
We describe a new method that permits quantification in the pmol to nmol range of three lyso-neutral glycosphingolipids (lyso-n-GSLs), glucosylsphingosine (GlcSph), galactosylsphingosine (GalSph), and lactosylsphingosine, in the same sample as neutral ...
Jacques Bodennec +2 more
doaj +1 more source
Synthesis and biological evaluation of novel PDMP analogues [PDF]
A new series of hybrid PDMP analogues, based both on PDMP and styryl analogues of natural ceramide, has been synthesized from D-serine. The synthetic route was developed such that future introduction of different aryl groups is straightforward ...
Boldin-Adamsky, Swetlana +5 more
core +1 more source
No evidence for substrate accumulation in Parkinson brains with GBA mutations [PDF]
To establish whether Parkinson's disease (PD) brains previously described to have decreased glucocerebrosidase activity exhibit accumulation of the lysosomal enzyme's substrate, glucosylceramide, or other changes in lipid ...
Gegg, ME +5 more
core +1 more source
Sphingolipids in human synovial fluid : a lipidomic study [PDF]
Articular synovial fluid (SF) is a complex mixture of components that regulate nutrition, communication, shock absorption, and lubrication. Alterations in its composition can be pathogenic. This lipidomic investigation aims to quantify the composition of
Kaesser, Ulrich +10 more
core +6 more sources
The glycosphingolipid composition of brain and visceral tissue from a patient with an unusual neurovisceral lipid storage disease, characterized by a lactosylceramide galactosyl hydrolase deficiency, was determined. Analyses of erythrocytes, plasma, bone
Glyn Dawson
doaj +1 more source
Several patients who had a progressive clinical course involving both the central and peripheral nervous systems have been reported, but the diagnostic marker has been remained uncertain.
Hitoki Nanaura +7 more
doaj +1 more source
Induced Pluripotent Stem Cells Derived from a CLN5 Patient Manifest Phenotypic Characteristics of Neuronal Ceroid Lipofuscinoses [PDF]
Neuronal ceroid lipofuscinoses (NCLs) are autosomal recessive progressive encephalopathies caused by mutations in at least 14 different genes. Despite extensive studies performed in different NCL animal models, the molecular mechanisms underlying ...
Blom, Tea +5 more
core +2 more sources
Glycosphingolipids from cultured astroblasts
The glycolipids of two clonal lines astroblasts, NN clone and C6 clone, were studied. Glucosylceramide and lactosylceramide were present at very low levels in both clones, but the most common myelin glycolipids galactosylceramide and sulfatide, were not ...
J Robert, G Rebel, P Mandel
doaj +1 more source
It is uncertain which β4-galactosyltransferase (β4GalT; gene name, B4galt), β4GalT-5 and/or β4GalT-6, is responsible for the production of lactosylceramide (LacCer) synthase, which functions in the initial step of ganglioside biosynthesis.
T. Yoshihara +14 more
semanticscholar +1 more source
Use of a glycolipid inhibitor to ameliorate renal cancer in a mouse model.
In a xenograft model wherein, live renal cancer cells were implanted under the kidney capsule in mice, revealed a 30-fold increase in tumor volume over a period of 26 days and this was accompanied with a 32-fold increase in the level of lactosylceramide (
Subroto Chatterjee +8 more
doaj +1 more source

