Results 11 to 20 of about 10,573 (148)

[29] Sphingolipid hydrolases and activator proteins

Biochimie, 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, Thomas Kolter, Konrad Sandhoff   +2 more
core   +7 more sources

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

Disease 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, Heike Schulze, Marie Paradis, Dominic Gosejacob, Cyrus Papan, Andrej Shevchenko, Olympia Ekaterina Psathaki, Achim Paululat, Melanie Thielisch, Konrad Sandhoff, Michael Hoch   +10 more
doaj   +2 more sources

The lysomal trafficking of sphingolipid activator proteins (SAPs) is mediated by sortilin [PDF]

The EMBO Journal, 2003
Most soluble lysosomal proteins bind the mannose 6-phosphate receptor (M6P-R) to be sorted to the lysosomes. However, the lysosomes of I-cell disease (ICD) patients, a condition resulting from a mutation in the phosphotransferase that adds mannose 6-phosphate to hydrolases, have near normal levels of several lysosomal proteins, including the ...
Stephane, Lefrancois, Jibin, Zeng, A Jacob, Hassan, Maryssa, Canuel, Carlos R, Morales   +4 more
openaire   +4 more sources

Sphingolipid activator proteins: proteins with complex functions in lipid degradation and skin biogenesis [PDF]

Glycobiology, 2001
Sphingolipid activator proteins (SAPs or saposins) are essential cofactors for the lysosomal degradation of membrane-anchored sphingolipids. Four of the five known proteins of this class, SAPs A--D, derive from a single precursor protein and show high homology, whereas the fifth protein, GM2AP, is larger and displays a different secondary structure ...
Schuette, C. G., Pierstorff, B., Huettler, S., Sandhoff, K.   +3 more
openaire   +4 more sources

Diverse Sphingolipid Species Harbor Different Effects on Ire1 Clustering [PDF]

, 2022
Endoplasmic reticulum (ER) function is dedicated to multiple essential processes in eukaryotes, including the processing of secretory proteins and the biogenesis of most membrane lipids.
Steuart, Robert F. L., Christopher M. Witham, Bieniawski, Mark A., Stevens, Kofi L.P., Bankaitis, Vytas A., Stevens, Kofi L. P., Mark A. Bieniawski, Vytas A. Bankaitis, Bankaitis, V.A., Witham, Christopher M., Robert F. L. Steuart, Kofi L. P. Stevens, Mousley, Carl J., Carl J. Mousley   +13 more
core   +1 more source

β-Hexosaminidase B and Sphingolipid Activator Proteins [PDF]

, 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

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

Journal 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, Stephen Duplock, Maria Fuller   +2 more
doaj   +1 more source

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

Journal 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, Bernadette Breiden, Matthew A. Adebayo, Jonathan O. Babalola, Günter Schwarzmann, Konrad Sandhoff   +5 more
doaj   +1 more source

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

Journal 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, Detlev Schindler, Jana Ledvinová, Bohuslav Černý, František Šmíd, Ernst Conzelmann   +5 more
doaj   +1 more source

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

Neurobiology 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, Sarita Hebbar, Dominik Schwudke, Christopher J.H. Elliott, Sean T. Sweeney   +4 more
doaj   +1 more source