Results 211 to 220 of about 2,765,821 (261)

Sphingolipid hydrolase activator proteins and their precursors

Biochemical and Biophysical Research Communications, 1989
Activator proteins for sphingolipid hydrolases (saposins) are small acidic, heat-stable glycoproteins that stimulate the hydrolysis of sphingolipids by lysosomal enzymes. The molecular mass of each stimulator is about 10 kDa, but glycosylated forms of higher mass exist too.
Akira Sano, Shû-Ichi Ueno, Yasuo Kakimoto   +2 more
exaly   +4 more sources

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

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 ...
K. Sandhoff, A. Klein
exaly   +3 more sources

Sphingolipid Activator Proteins (SAPs) in Neuronal Ceroid Lipofuscinoses (NCL)

Neuropediatrics, 1997
Based on the predominant component of the storage material the neuronal ceroid lipofuscinoses (NCL) can be divided into two categories: one storing mitochondrial ATP synthase subunit c and the other storing sphingolipid activator proteins (SAPs). The latter group is represented by the human infantile NCL (INCL), a congenital ovine NCL, and a canine NCL.
J, Tyynelä, J, Suopanki, M, Baumann, M, Haltia   +3 more
openaire   +3 more sources

Stimulation of Acid Sphingomyelinase Activity by Lysosomal Lipids and Sphingolipid Activator Proteins

Biological Chemistry, 2001
Acid sphingomyelinase is a water-soluble, lysosomal glycoprotein that catalyzes the degradation of membrane-bound sphingomyelin into phosphorylcholine and ceramide. Sphingomyelin itself is an important component of the extracellular leaflet of various cellular membranes.
T, Linke, G, Wilkening, S, Lansmann, H, Moczall, O, Bartelsen, J, Weisgerber, K, Sandhoff   +6 more
openaire   +3 more sources

Determination of saposin proteins (sphingolipid activator proteins) in human tissues

Analytical Biochemistry, 1990
Saposins are small glycoproteins which are required for sphingolipid hydrolysis by lysosomal hydrolases. Each saposin (A, B, C, and D) stimulates a different enzymatic activity. A new simple HPLC method to determine the levels of saposins A, C, and D in tissue was developed. Tissues were homogenized in 20 vol of water, boiled, and centrifuged.
S, Morimoto, Y, Yamamoto, J S, O'Brien, Y, Kishimoto   +3 more
openaire   +3 more sources

Sphingolipid Activator Proteins

2009
Glycosphingolipids (GSLs) are amphiphilic constituents of the outer leaflet of eukaryotic plasma membranes. In vivo degradation of GSLs takes place predominantly in the lysosome by the stepwise release of monosaccharide units from the nonreducing end of the oligosaccharide chain via specific exohydrolases (Fig. 1).
J. Matsuda
openaire   +2 more sources

IMMUNOLOGICAL STUDIES ON SPHINGOLIPID ACTIVATOR PROTEINS IN THE NEURONAL CEROID-LIPOFUSCINOSES

Gerontology, 2009
The neuronal ceroid-lipofuscinoses constitute an important group of progressive encephalopathies leading to severe psychomotor retardation, blindness, and early death. They are characterized by accumulation of autofluorescent, electron-dense storage bodies within the cytoplasm of neurons and many other cell types.
M, Haltia, J, Tyynelä, M, Baumann, M, Henseler, K, Sandhoff   +4 more
openaire   +3 more sources

Sphingolipid activator proteins in the neuronal ceroid-lipofuscinoses: an immunological study

Acta Neuropathologica, 1995
The molecular defects underlying neuronal ceroid-lipofuscinoses (NCL) are still unknown. However, more data exist on the composition of the hydrophobic storage material characteristic of NCL. Accumulation of subunit c of the mitochondrial ATP synthase has been shown in most forms of human NCL with the exception of the infantile NCL (INCL) for which we ...
Konrad Sandhoff, Jaana Tyynelä, Matti Haltia   +2 more
exaly   +3 more sources

Sphingolipid activator proteins in a human hereditary renal disease with deposition of disialogangliosides

The Histochemical Journal, 1996
Congenital nephrotic syndrome of the Finnish type is a recessively inherited renal disease with glomerular deposits of the disialoganglioside O-acetyl-GD3. Sphingolipid activator proteins (saposins) stimulate the degradation of glycosphingolipids by lysosomal enzymes, and defects in saposins cause accumulation of substrate lipids in the affected ...
A, Haltia, M L, Solin, H, Jalanko, C, Holmberg, A, Miettinen, H, Holthöfer   +5 more
openaire   +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
Yu-Teh Li, Su-Chen Li
openaire   +2 more sources