Results 81 to 90 of about 11,067 (122)

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 ...
Azusa Yoneshige, Kunihiko Suzuki
exaly   +3 more sources

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.
A, Sano, T, Hineno, T, Mizuno, K, Kondoh, S, Ueno, Y, Kakimoto, K, Inui   +6 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

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   +2 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   +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

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 ...
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