Results 221 to 230 of about 2,765,821 (261)

Pathological study of mice with total deficiency of sphingolipid activator proteins (SAP knockout mice)

Acta Neuropathologica, 1998
Nobuya Fujita
exaly   +2 more sources

Sphingolipid activator proteins (SAPS) are stored together with glycosphingolipids in the infantile neuronal ceroid-lipofuscinosis (INCL)

American Journal of Medical Genetics Part A, 1995
Konrad Sandhoff, Jaana Tyynelä, M Haltia   +2 more
exaly   +2 more sources

Physiological relevance of sphingolipid activator proteins in cultured human fibroblasts

Biochimie, 2003
Bernadette Breiden, Konrad Sandhoff
exaly   +2 more sources

Sphingolipid Metabolism: Sphingoid Analogs, Sphingolipid Activator Proteins, and the Pathology of the Cella

Annals of the New York Academy of Sciences, 1998
Konrad Sandhoff
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 [PDF]

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.
Konrad Sandhoff, R Kase, H Sakuraba
exaly   +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

Metabolism of Gangliosides: Topology, Pathobiochemistry, and Sphingolipid Activator Proteins

Current Topics in Membranes, 1994
Konrad Sandhoff
exaly   +2 more sources

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 ...
H, Shigematsu, S, Morimoto, Y, Kishimoto, S, Weiler, J, Tomich, J, Barranger, M, Shinohara, A M, Yeager, J S, O'Brien   +8 more
openaire   +2 more sources

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