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Lipid composition of the normal human brain: gray matter, white matter, and myelin.
TLDR
The extra-myelin portion of white matter had a lipid composition that was very similar to that of myelin, but quite different from that of gray matter.
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Saposins: structure, function, distribution, and molecular genetics.
TLDR
The physiological significance of saposins is underlined by their accumulation in tissues of lysosomal storage disease patients and the occurrence of sphingolipidosis due to mutations in the prosaposin gene.
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Fucosidosis revisited: a review of 77 patients.
TLDR
A review of fucosidosis, compiling data from published reports and an international questionnaire survey, found evidence for the existence of clinical heterogeneity with a rapidly progressive type I and a slowly progressive type II fucOSidosis as suggested in the literature and found a wide continuous clinical spectrum.
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Fatty acid and fatty aldehyde composition of the major brain lipids in normal human gray matter, white matter, and myelin.
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Saposin proteins: structure, function, and role in human lysosomal storage disorders
TLDR
Characterization of these four activator proteins, two of which were recently discovered, and their importance in human health and disease are reviewed, including their role in human lysosomal storage disorders.
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Cloning and expression of human arylsulfatase A.
TLDR
The cDNA sequence shows no homology to any of the known sequences of lysosomal enzymes but a 35% identity to human steroid sulfatase, which indicates that proteolytic processing of arylsulfatase A is confined to the cleavage of the signal peptide.
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Prosaptide prevents paclitaxel neurotoxicity.
TLDR
It is suggested that prosaptide prevents the neurotoxic effects of paclitaxel while not interfering with its anti-tumor activity.
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Sialidosis: a review of human neuraminidase deficiency.
TLDR
The clinical phenotype of primary human neuraminidase deficiency is reviewed and the term "sialidosis" is employed here to describe patients with primary neuraminIDase deficiency and should be reserved exclusively for this condition.
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Identification of prosaposin as a neurotrophic factor.
TLDR
Results indicate that prosaposin and saposin C are neurotrophic factors which initiate signal transduction by binding to a high-affinity receptor that induces protein phosphorylation.
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Prosaptide activates the MAPK pathway by a G-protein-dependent mechanism essential for enhanced sulfatide synthesis by Schwann cells.
TLDR
Data demonstrate that TX14(A) uses a pertussis toxin-sensitive G-protein pathway to activate ERKs, which is essential for enhanced sulfatide synthesis in Schwann cells.
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