Results 101 to 110 of about 10,573 (148)

Stimulation of lysosomal sphingomyelin degradation by sphingolipid activator proteins

open access: yesChemistry and Physics of Lipids, 1999
Lysosomal breakdown of glycosphingolipids with short hydrophilic carbohydrate headgroups is achieved by the simultaneous action of specific hydrolases and sphingolipid activator proteins (SAPs). Activator proteins are considered to facilitate the enzyme/substrate interaction between water-soluble enzymes and membrane-bound substrates.
Klaus Ferlinz   +2 more
exaly   +4 more sources

Sphingolipid activator proteins in the neuronal ceroid-lipofuscinoses: an immunological study

open access: yesActa 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 ...
Marc Baumann   +2 more
exaly   +4 more sources
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Activator proteins and topology of lysosomal sphingolipid catabolism

Lipids and Lipid Metabolism, 1992
The lysosomal degradation of several sphingolipids by acid hydrolases is dependent on small non-enzymic cofactors, called sphingolipid activator proteins some of which have been identified as sphingolipid binding proteins. This review summarizes the information available on the structure, function, biosynthesis, gene organization and pathobiochemistry ...
Konrad Sandhoff, W Fürst
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.
Akira Sano   +2 more
exaly   +3 more sources

Sphingolipid Activator Proteins

open access: yes, 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).
Junko Matsuda
openaire   +2 more sources

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