Results 171 to 180 of about 10,624 (209)

PRINCIPLES OF LYSOSOMAL MEMBRANE DIGESTION: Stimulation of Sphingolipid Degradation by Sphingolipid Activator Proteins and Anionic Lysosomal Lipids

Annual Review of Cell and Developmental Biology, 2005
Sphingolipids and glycosphingolipids are membrane components of eukaryotic cell surfaces. Their constitutive degradation takes place on the surface of intra-endosomal and intra-lysosomal membrane structures. During endocytosis, these intra-lysosomal membranes are formed and prepared for digestion by a lipid-sorting process during which their ...
Thomas Kolter, Konrad Sandhoff
exaly   +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, J Tyynelä, Matti Haltia   +2 more
exaly   +3 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

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

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 (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

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