Results 181 to 190 of about 1,668,348 (220)

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 ...
Konrad Sandhoff
exaly   +3 more sources

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, Jaana Tyynelä, Matti Haltia   +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 L. 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.
Thomas Kolter, Konrad Sandhoff, Ryoichi Kase   +2 more
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

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

Intracellular trafficking of glycosphingolipids: Role of sphingolipid activator proteins in the topology of endocytosis and lysosomal digestion [PDF]

FEBS Letters, 1994
Glycosphingolipids (GSL) are components of the outer leaflet of the plasma membrane (PM) of vertebrate tissues. Our current knowledge of GSL metabolism and their intracellular traffic has been derived from metabolic studies but the exact mechanisms by ...
Konrad Sandhoff
exaly   +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