Results 191 to 200 of about 1,665,622 (222)

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   +2 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   +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   +2 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).
openaire   +1 more source

Products of Sphingolipid Catabolism Block Activation of the p21-Activated Protein Kinases in Neutrophils

The Journal of Immunology, 1998
Abstract Neutrophils stimulated with the chemoatttractant FMLP are known to exhibit a rapid and transient activation of two p21-activated protein kinases (Paks) with molecular masses of approximately 63 and 69 kDa. Paks can be detected by their ability to undergo renaturation and catalyze the phosphorylation of a peptide substrate ...
J P, Lian, R, Huang, D, Robinson, J A, Badwey   +3 more
openaire   +2 more sources

Human sphingolipid activator protein-1 and sphingolipid activator protein-2 are encoded by the same gene.

Journal of molecular neuroscience : MN, 1990
Mixed oligonucleotide primers complementary to the translation product of the sphingolipid activator protein (SAP)-2 were used to generate a 144-base pair (bp) complementary DNA (cDNA). This cDNA probe was used to isolate a 2,649-nucleotide-long cDNA that was sequenced and found to contain coding sequences for two known activators of lysosomal enzymes,
O, Reiner, O, Dagan, M, Horowitz
openaire   +1 more source

The function of sphingolipids in the nervous system: lessons learnt from mouse models of specific sphingolipid activator protein deficiencies

Journal of Neurochemistry, 2007
AbstractWe have generated specific saposin A and D deficient mouse mutants by the gene targeting technology. Saposin A deficient mice showed the clinical, biochemical and pathological phenotype of a chronic form of globoid cell leukodystrophy (Krabbe disease) establishing that saposin A is essential for in vivo degradation of galactosylceramide ...
Junko, Matsuda, Azusa, Yoneshige, Kunihiko, Suzuki   +2 more
openaire   +2 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 ...
Sandhoff, K., Klein, A., K. Sandhoff, A. Klein   +3 more
exaly   +2 more sources

Biochemical, immunological, and structural studies on a sphingolipid activator protein (SAP-1)

Archives of Biochemistry and Biophysics, 1984
Sphingolipid activator protein-1 (SAP-1) is a glycoprotein found in human tissue extracts that stimulates the enzymatic hydrolysis of at least two glycosphingolipids, including GM1 ganglioside and sulfatide. The ability of purified SAP-1 to stimulate GM1 ganglioside hydrolysis by extracts of cultured fibroblasts from patients with beta-galactosidase ...
K, Inui, D A, Wenger
openaire   +2 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   +2 more sources