Results 191 to 200 of about 1,668,348 (220)

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
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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).
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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
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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
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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
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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
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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
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Molecular cloning of the sphingolipid activator protein — 1 (SAP - 1), the sulfatide sulfatase activator

Biochemical and Biophysical Research Communications, 1986
A cDNA coding for SAP-1 was isolated from a lambda gt11 human hepatoma expression library using polyclonal antibodies raised against human SAP-1. Three positive clones were isolated with inserts of approximately 0.3 Kb (S1.1), 2 Kb (S1.2) and 2.2 Kb (S-1.3). The latter 2 contained an internal EcoRI site.
N, Dewji, D, Wenger, S, Fujibayashi, M, Donoviel, F, Esch, F, Hill, J S, O'Brien   +6 more
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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
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[Disorders of sphingolipid activator proteins].

Nihon rinsho. Japanese journal of clinical medicine, 1996
Small-molecular nonenzymatic glycoproteins are necessary for degradation of sphingolipids in lysosomes. GM2 activator encoded by a gene on chromosome 5 is essential for hydrolysis of ganglioside GM2 and its asialo derivative. Mutations of this gene cause Tay-Sachs disease-like clinical phenotype (GM2-gangliosidosis AB variant).
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