Results 31 to 40 of about 2,765,821 (261)

Role of endosomal membrane lipids and NPC2 in cholesterol transfer and membrane fusion[S]

open access: yesJournal of Lipid Research, 2010
We examined the effect of Niemann-Pick disease type 2 (NPC2) protein and some late endosomal lipids [sphingomyelin, ceramide and bis(monoacylglycero)phosphate (BMP)] on cholesterol transfer and membrane fusion.
Misbaudeen Abdul-Hammed   +5 more
doaj   +1 more source

β-Hexosaminidase B and Sphingolipid Activator Proteins [PDF]

open access: yes, 2004
Titelblatt Inhaltsverzeichnis Zusammenfassung Danksagung Abkürzungen 1 Einleitung 1 1.1 Erbliche Stoffwechselstörungen 1 1.2 Glykosphingolipide 3 1.3 Glykosphingolipid ...
Maier, Timm
core   +1 more source

Characterization of Drosophila Saposin-related mutants as a model for lysosomal sphingolipid storage diseases

open access: yesDisease Models & Mechanisms, 2017
Sphingolipidoses are inherited diseases belonging to the class of lysosomal storage diseases (LSDs), which are characterized by the accumulation of indigestible material in the lysosome caused by specific defects in the lysosomal degradation machinery ...
Julia Sellin   +10 more
doaj   +1 more source

The essential neutral sphingomyelinase is involved in the trafficking of the variant surface glycoprotein in the bloodstream form of Trypanosoma brucei [PDF]

open access: yes, 2010
Sphingomyelin is the main sphingolipid in Trypanosoma brucei, the causative agent of African sleeping sickness. In vitro and in vivo characterization of the T.
Smith, Terry K.   +2 more
core   +1 more source

Degradation of blood group A glycolipid A-6-2 by normal and mutant human skin fibroblasts

open access: yesJournal of Lipid Research, 1998
The degradation of blood group glycolipid A-6-2 (GalNAc(α1→3)[Fucα1→2]Gal(β1→4)GlcNAc(β1→3)Gal (β1→4)Glc(β1→1′)Cer, IV2-α-fucosyl-IV3-α-N-acetylgalactosaminylneolactotetraosylceramide), tritium-labeled in its ceramide moiety, was studied in situ, in skin
Befekadu Asfaw   +5 more
doaj   +1 more source

A saposin deficiency model in Drosophila: Lysosomal storage, progressive neurodegeneration and sensory physiological decline

open access: yesNeurobiology of Disease, 2017
Saposin deficiency is a childhood neurodegenerative lysosomal storage disorder (LSD) that can cause premature death within three months of life.
Samantha J. Hindle   +4 more
doaj   +1 more source

Regulation of Mitogen-Activated Protein Kinases by Sphingolipid Products in Oligodendrocytes [PDF]

open access: yesThe Journal of Neuroscience, 1999
Sphingolipid products such as ceramide (cer), sphingosine (sph), and sphingosine-1-phosphate (SPP) are implicated in the regulation of cell growth and apoptosis. We have recently shown that cer, sph, and SPP differentially modulate ionic events in cultured oligodendrocytes (OLGs).
H, Hida   +3 more
openaire   +2 more sources

Sphingolipid-Transporting Proteins as Cancer Therapeutic Targets [PDF]

open access: yes, 2019
This article was supported by the German Research Foundation (DFG) and the Open Access Publication Fund of Humboldt-Universität zu Berlin.The understanding of the role of sphingolipid metabolism in cancer has tremendously increased in the past ten years.
Arenz, Christoph   +9 more
core   +1 more source

Next-Generation Sequencing Analysis Reveals Novel Pathogenic Variants in Four Chinese Siblings With Late-Infantile Neuronal Ceroid Lipofuscinosis

open access: yesFrontiers in Genetics, 2019
Neuronal Ceroid Lipofuscinoses (NCLs) are progressive degenerative diseases mainly affect brain and retina. They are characterized by accumulation of autofluorescent storage material, mitochondrial ATPase subunit C, or sphingolipid activator proteins A ...
Xiao-Tun Ren   +8 more
doaj   +1 more source

Orm family proteins mediate sphingolipid homeostasis [PDF]

open access: yes, 2010
Despite the essential roles of sphingolipids both as structural components of membranes and critical signalling molecules, we have a limited understanding of how cells sense and regulate their levels.
Ruedi Aebersold   +28 more
core   +1 more source

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