Reconstruction of Molecular Interaction Patterns from Endolysosomes in Ceramide-Depleted Cells. [PDF]
Feng Y +7 more
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Congenital Disorders of Glycosphingolipid Biosynthesis: Ultrarare Severe Syndromes or Relatively Frequent Mild Neurocognitive Illnesses? [PDF]
Montavoci L +3 more
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Intestinal bacteria trigger a hibernation-like state in homotherms via the gut-brain axis. [PDF]
Bao Z +10 more
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Sphingolipid metabolism-related genes B4GALNT1 and CERS4 as prognostic biomarkers in lung adenocarcinoma. [PDF]
Jeon J +6 more
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Genetic Architecture of Synaptic Failure in Dementia with Lewy Bodies: From α-Synuclein Proteoforms to GBA1-Mediated Plasticity Deficits. [PDF]
Bougea A.
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Ceramide kinase/ceramide 1-phosphate signaling regulates LC3B expression and autophagosome formation. [PDF]
Funou H +7 more
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Activator proteins and topology of lysosomal sphingolipid catabolism
Lipids and Lipid Metabolism, 1992The 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
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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
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Stimulation of lysosomal sphingomyelin degradation by sphingolipid activator proteins
Chemistry and Physics of Lipids, 1999Lysosomal 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.
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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 ...
John S O'Brien, Hidenari Shigematsu
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