Results 21 to 30 of about 1,124 (173)
Structure of saposin A lipoprotein discs [PDF]
Proceedings of the National Academy of Sciences, 2012 The saposins are small, membrane-active proteins that exist in both soluble and lipid-bound states. Saposin A has roles in sphingolipid catabolism and transport and is required for the breakdown of galactosylceramide by β-galactosylceramidase. In the absence of lipid, saposin A adopts a closed monomeric apo conformation typical of this family. To study
Konstantin, Popovic +3 more
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Saposin D: A sphingomyelinase activator [PDF]
Biochemical and Biophysical Research Communications, 1988 Saposin D, a newly discovered heat-stable, 10 kDa glycoprotein, was isolated from Gaucher spleen and purified to homogeneity. Chemical sequencing from its amino terminus demonstrated colinearity between its amino acid sequence and the deduced amino acid sequence of the fourth domain of prosaposin, the precursor of saposin proteins.
Morimoto, Satoshi +3 more
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Tissue-specific effects of saposin A and saposin B on glycosphingolipid degradation in mutant mice [PDF]
Human Molecular Genetics, 2013 Individual saposin A (A-/-) and saposin B (B-/-)-deficient mice show unique phenotypes caused by insufficient degradation of myelin-related glycosphingolipids (GSLs): galactosylceramide and galactosylsphingosine and sulfatide, respectively. To gain insight into the interrelated functions of saposins A and B, combined saposin AB-deficient mice (AB ...
Ying, Sun +10 more
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Modelling saposin deficiency in Drosophila: progressive neurodegeneration, storage and physiological decline [PDF]
, 2010 Saposin deficiency is a lysosomal storage disorder (LSD) characterised by the lysosomal accumulation of sphingolipids. The disorder is caused by mutations in the prosaposin gene, which encodes 4 activator proteins: saposins A - D.
Hindle, Samantha Hindle
core +6 more sources
Lysosomal Storage Diseases: Heterogeneous Group of Disorders [PDF]
BioImpacts, 2013 The name of lysosomal storage diseases stems from the fact that in this category of disorders specific undegraded materials are stored in the lysosomes.
David A. Wenger +2 more
doaj +1 more source
Role of sphingolipids in the transport of prosaposin to the lysosomes
Journal of Lipid Research, 1999 Prosaposin is the precursor of four lysosomal saposins that promote the degradation of glycosphingolipids (GSLs) by acidic hydrolases. GSLs contain a hydrophobic ceramide moiety, which acts as a membrane anchor, and a hydrophilic oligosaccharide chain ...
Stephane Lefrancois +4 more
doaj +1 more source
Structure of human saposin A at lysosomal pH [PDF]
Acta Crystallographica Section F Structural Biology Communications, 2015 The saposins are essential cofactors for the normal lysosomal degradation of complex glycosphingolipids by acid hydrolase enzymes; defects in either saposin or hydrolase function lead to severe metabolic diseases. Saposin A (SapA) activates the enzyme β-galactocerebrosidase (GALC), which catalyzes the breakdown of β-D-galactocerebroside, the principal ...
Hill, Chris H +2 more
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The role of saposin C in Gaucher disease [PDF]
Molecular Genetics and Metabolism, 2012 Saposin C is one of four homologous proteins derived from sequential cleavage of the saposin precursor protein, prosaposin. It is an essential activator for glucocerebrosidase, the enzyme deficient in Gaucher disease. Gaucher disease is a rare autosomal recessive lysosomal storage disorder caused by mutations in the GBA gene that exhibits vast ...
Rafael J, Tamargo +3 more
openaire +2 more sources
Neurobiology of Disease, 2017 Saposin deficiency is a childhood neurodegenerative lysosomal storage disorder (LSD) that can cause premature death within three months of life.
Samantha J. Hindle +4 more
doaj +1 more source