Results 181 to 190 of about 1,665,622 (222)
Some of the next articles are maybe not open access.
Sphingolipid hydrolase activator proteins and their precursors
Biochemical and Biophysical Research Communications, 1989Activator 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.
Akira Sano +2 more
exaly +3 more sources
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 ...
Konrad Sandhoff
exaly +3 more sources
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 ...
Konrad Sandhoff
exaly +3 more sources
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
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, 1995The 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 ...
Marc Henrik Baumann +2 more
exaly +3 more sources
Quantification of mRNAs encoding proteins of the glycosphingolipid catabolism in mouse models of GM2 gangliosidoses and sphingolipid activator protein precursor (prosaposin) deficiency [PDF]
Biochimica Et Biophysica Acta - Molecular Basis of Disease, 2000 We have investigated the mRNA amounts of six lysosomal proteins (β-hexosaminidase α- and β-subunit, sphingolipid activator protein precursor, GM2 activator protein, lysosomal sialidase, β-glucocerebrosidase) involved in the degradation of ...
Richard Proia, 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
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 +2 more
openaire +2 more sources
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 +2 more
openaire +2 more sources
Metabolism of Gangliosides: Topology, Pathobiochemistry, and Sphingolipid Activator Proteins
Current Topics in Membranes, 1994Konrad Sandhoff
exaly +2 more sources
Saposins (Sphingolipid Activator Proteins) in the Twitcher Mutant Mouse
Journal of Neurochemistry, 1990Abstract: 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 +8 more
openaire +2 more sources
Determination of saposin proteins (sphingolipid activator proteins) in human tissues
Analytical Biochemistry, 1990Saposins 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 +3 more
openaire +2 more sources