Zobrazeno 1 - 10
of 28
pro vyhledávání: '"Dori Pelled"'
Autor:
Dori Pelled, Selena Trajkovic-Bodennec, Emyr Lloyd-Evans, Ellen Sidransky, Raphael Schiffmann, Anthony H. Futerman
Publikováno v:
Neurobiology of Disease, Vol 18, Iss 1, Pp 83-88 (2005)
Gaucher disease is an inherited metabolic disorder caused by defective activity of the lysosomal enzyme, glucocerebrosidase, resulting in accumulation of the lipids, glucosylceramide (GlcCer), and glucosylsphingosine (GlcSph). Little is known about t
Externí odkaz:
https://doaj.org/article/e6d0cd9d057948a1880b81ef838919f6
Autor:
Yonatan Ganor, Hadassa Goldberg-Stern, Dina Amrom, Tally Lerman-Sagie, Vivian I. Teichberg, Dori Pelled, Anthony H. Futerman, Bruria Ben Zeev, Michael Freilinger, Denis Verheulpen, Patrick Van Bogaert, Mia Levite
Publikováno v:
Clinical and Developmental Immunology, Vol 11, Iss 3-4, Pp 241-252 (2004)
Purpose: Elucidating the potential contribution of specific autoantibodies (Ab's) to the etiology and/or pathology of some human epilepsies. Methods: Six epilepsy patients with Rasmussen's encephalitis (RE) and 71 patients with other epilepsies we
Externí odkaz:
https://doaj.org/article/a1a0b92e91d64c2386b1e8c47398e41c
Publikováno v:
Journal of Lipid Research, Vol 44, Iss 1, Pp 218-226 (2003)
Methods for isolation of neutral lysoglycosphingolipids (n-lys20) such as glucosylsphingosine and galactosylsphingosine normally involve mild alkaline or acid hydrolysis followed by multiple chromatography steps, yielding relatively low recoveries of
Externí odkaz:
https://doaj.org/article/762e849dbd7d4954b5a4d570475858d1
Publikováno v:
Journal of Neurochemistry. 72:1139-1145
Lesch-Nyhan syndrome is a pediatric metabolic-neurological syndrome caused by the X-linked deficiency of the purine salvage enzyme hypoxanthine-guanine phosphoribosyltransferase (HGPRT). The cause of the metabolic consequences of HGPRT deficiency has
Publikováno v:
The American Journal of Clinical Nutrition. 84:1534-1542
Background: Fish-oil fatty acid esters of plant sterols (FO-PS) were shown to have hypotriglyceridemic and hypocholesterolemic properties in animal models. Objective: The objective of the study was to evaluate the hypolipidemic effects of FO-PS suppl
Autor:
Selena Trajkovic-Bodennec, Raphael Schiffmann, Anthony H. Futerman, Ellen Sidransky, Emyr Lloyd-Evans, Dori Pelled
Publikováno v:
Neurobiology of Disease, Vol 18, Iss 1, Pp 83-88 (2005)
Gaucher disease is an inherited metabolic disorder caused by defective activity of the lysosomal enzyme, glucocerebrosidase, resulting in accumulation of the lipids, glucosylceramide (GlcCer), and glucosylsphingosine (GlcSph). Little is known about t
Publikováno v:
Biochemical Journal. 375:561-565
Recently, we demonstrated that the GSL (glycosphingolipid), GlcCer (glucosylceramide), modulates Ca2+ release from intracellular stores and from microsomes by sensitizing the RyaR (ryanodine receptor), a major Ca2+-release channel of the endoplasmic
Autor:
Christian Riebeling, Dori Pelled, Frances M. Platt, Mylvaganam Jeyakumar, Emyr Lloyd-Evans, Anthony H. Futerman
Publikováno v:
Journal of Biological Chemistry. 278:29496-29501
Gangliosides are found at high levels in neuronal tissues where they play a variety of important functions. In the gangliosidoses, gangliosides accumulate because of defective activity of the lysosomal proteins responsible for their degradation, usua
Publikováno v:
Neuropathology and Applied Neurobiology. 29:341-349
Sandhoff disease is a lysosomal storage disease in which ganglioside GM2 accumulates because of a defective beta-subunit of beta-hexosaminidase. This disease is characterized by neurological manifestations, although the pathogenic mechanisms leading
Autor:
Aviv de-Morgan, Christian Riebeling, Dori Pelled, Anthony H. Futerman, Jacques Bodennec, Emyr Lloyd-Evans, Raphael Schiffmann, Helen Waller
Publikováno v:
Journal of Biological Chemistry. 278:23594-23599
We recently demonstrated that elevation of intracellular glucosylceramide (GlcCer) levels results in increased functional Ca2+ stores in cultured neurons, and suggested that this may be due to modulation of ryanodine receptors (RyaRs) by GlcCer (Kork