Results 11 to 20 of about 1,761,902 (161)

Selective reduction of bis(monoacylglycero)phosphate ameliorates the storage burden in a THP-1 macrophage model of Gaucher disease

open access: yesJournal of Lipid Research, 2013
Bis(monoacylglycero)phosphate (BMP) assists lysosomal function by facilitating interaction of hydrolases and activator proteins with sphingolipid substrates.
Leanne K. Hein   +2 more
doaj   +1 more source

Functional analysis of TOR complex 2 and its control of sphingolipid biosynthesis in "Saccharomyces cerevisiae" [PDF]

open access: yes, 2006
sphingoid base, one of the intermediate of sphingolipids metabolites, plays a key role as a signaling molecule which activate PKH kinases and its substrates YPKs and PKC1 (Casamayor, Torrance et al. 1999; Friant, Lombardi et al.
Shioda, Ryo
core   +1 more source

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

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

[29] Sphingolipid hydrolases and activator proteins

open access: yesBiochimie, 2000
The physiological degradation of several membrane-bound glycosphingolipids (GSLs) by water-soluble lysosomal exohydrolases requires the assistance of sphingolipid activator proteins (SAPs). Four of these SAPs are synthesized from a single precursor protein (prosaposin).
Uwe Bierfreund   +2 more
  +7 more sources

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

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.   +4 more
core   +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

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