Results 111 to 120 of about 10,573 (148)

Saposins (Sphingolipid Activator Proteins) in the Twitcher Mutant Mouse

Journal of Neurochemistry, 1990
Abstract: The twitcher mutant mouse, the animal model of Krabbe disease (human globoid cell leukodystrophy), is characterized by apparent deficiency of galactosylceramide β‐galactosidase activity. Saposin A and C, the heat‐stable small sphingolipid activator glycoproteins, stimulate the activity of galactosylceramide β‐galactosidase as well as ...
Hidenari Shigematsu, Yasuo Kishimoto, John S O'Brien   +2 more
exaly   +3 more sources

Neuronal sphingolipidoses: Membrane lipids and sphingolipid activator proteins regulate lysosomal sphingolipid catabolism

Biochimie, 2016
Glycosphingolipids and sphingolipids of cellular plasma membranes (PMs) reach luminal intra-lysosomal vesicles (LVs) for degradation mainly by pathways of endocytosis. After a sorting and maturation process (e.g. degradation of sphingomyelin (SM) and secretion of cholesterol), sphingolipids of the LVs are digested by soluble enzymes with the help of ...
Konrad Sandhoff
exaly   +3 more sources

PRINCIPLES OF LYSOSOMAL MEMBRANE DIGESTION: Stimulation of Sphingolipid Degradation by Sphingolipid Activator Proteins and Anionic Lysosomal Lipids

Annual Review of Cell and Developmental Biology, 2005
Sphingolipids and glycosphingolipids are membrane components of eukaryotic cell surfaces. Their constitutive degradation takes place on the surface of intra-endosomal and intra-lysosomal membrane structures. During endocytosis, these intra-lysosomal membranes are formed and prepared for digestion by a lipid-sorting process during which their ...
Thomas Kolter, Konrad Sandhoff
exaly   +3 more sources

12 Activator Proteins for Sphingolipid Hydrolysis

, 1983
Publisher Summary This chapter focuses on the various activator proteins used for the sphingolipid hydrolysis. Mraz et al. found that human hepatic activator protein that was specific for stimulating the hydrolysis of galactosylceramide sulfate by human enzyme could also stimulate the same reaction catalyzed by acidic sulfatases isolated from various
Su-Chen Li, Yu-Teh Li
openaire   +2 more sources

Distribution of Saposins (Sphingolipid Activator Proteins) in Tissues of Lysosomal Storage Disease Patients

Journal of Molecular Neuroscience, 1992
Saposins are a group of small glycoproteins derived from a single precursor protein, prosaposin. Each of the four saposins are involved in lysosomal hydrolysis of various sphingolipids. Our recent investigations have shown that saposins accumulate in tissues of several lysosomal storage diseases patients, including those with Tay-Sachs disease and ...
M, Tayama, J S, O'Brien, Y, Kishimoto
openaire   +3 more sources

Quantification of mRNAs encoding proteins of the glycosphingolipid catabolism in mouse models of GM2 gangliosidoses and sphingolipid activator protein precursor (prosaposin) deficiency

Biochimica Et Biophysica Acta - Molecular Basis of Disease, 2000
We have investigated the mRNA amounts of six lysosomal proteins (β-hexosaminidase α- and β-subunit, sphingolipid activator protein precursor, GM2 activator protein, lysosomal sialidase, β-glucocerebrosidase) involved in the degradation of ...
Richard L Proia, Kunihiko Suzuki, Konrad Sandhoff   +2 more
exaly   +2 more sources

Only sphingolipid activator protein B (SAP-B or saposin B) stimulates the degradation of globotriaosylceramide by recombinant human lysosomal α-galactosidase in a detergent-free liposomal system

FEBS Letters, 1996
The degradation of globotriaosylceramide (GbOse3Cer) by insect-cell derived recombinant human α-galactosidase (EC 3.2.1.22) was carried out in a detergent-free liposomal system in order to mimic intralysosomal conditions.
Ryoichi Kase, Thomas Kolter, Konrad Sandhoff   +2 more
exaly   +2 more sources

Intracellular trafficking of glycosphingolipids: Role of sphingolipid activator proteins in the topology of endocytosis and lysosomal digestion

FEBS Letters, 1994
Glycosphingolipids (GSL) are components of the outer leaflet of the plasma membrane (PM) of vertebrate tissues. Our current knowledge of GSL metabolism and their intracellular traffic has been derived from metabolic studies but the exact mechanisms by ...
Konrad Sandhoff
exaly   +2 more sources

Saposin C and Other Sphingolipid Activator Proteins

, 2006
Thomas Kolter, Konrad Sandhoff, Silvia Locatelli Hoops   +2 more
openaire   +2 more sources

Sphingolipid Metabolism: Sphingoid Analogs, Sphingolipid Activator Proteins, and the Pathology of the Cell^a

Annals of the New York Academy of Sciences, 1998
ABSTRACT: Sphingolipid metabolism and function was investigated using sphingoid analogs, cells from human sphingolipidoses patients, and knockout animals. Treatment of primary cultured murine cerebellar cells with the structurally modified sphingosine base cis‐4‐methylsphingosine resulted in decreased sphingolipid biosynthesis accompanied by ...
K, Sandhoff, T, Kolter, G, Van Echten-Deckert   +2 more
openaire   +2 more sources