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Molecular basis of different forms of metachromatic leukodystrophy.
BACKGROUND Metachromatic leukodystrophy is an autosomal recessive inherited lysosomal storage disorder caused by a deficiency of arylsulfatase A. Three forms of the disease can be distinguishedExpand
Crystal structure of saposin B reveals a dimeric shell for lipid binding
Saposin B is a small, nonenzymatic glycosphingolipid activator protein required for the breakdown of cerebroside sulfates (sulfatides) within the lysosome. The protein can extract target lipids fromExpand
Two new arylsulfatase A (ARSA) mutations in a juvenile metachromatic leukodystrophy (MLD) patient.
Fragments of the arylsulfatase A (ARSA) gene from a patient with juvenile-onset metachromatic leukodystrophy (MLD) were amplified by PCR and ligated into MP13 cloning vectors. Clones hybridizing withExpand
Arylsulfatase a is present on the pig sperm surface and is involved in sperm-zona pellucida binding.
We have previously described the affinity of a pig sperm surface protein, P68, to mammalian zonae pellucidae (ZP). In this report, we identified P68 as arylsulfatase A (AS-A) based on the presence ofExpand
Isolation, characterization, and proteolysis of human prosaposin, the precursor of saposins (sphingolipid activator proteins).
Prosaposin contains separate domains in tandem for four saposins, A, B, C, and D. These mature saposins are produced by limited proteolysis of prosaposin. They are involved in lysosomal hydrolysis ofExpand
Coding of two sphingolipid activator proteins (SAP-1 and SAP-2) by same genetic locus.
Several complementary DNAs (cDNAs) coding for sphingolipid activator protein-2 (SAP-2) were isolated from a lambda gt-11 human hepatoma library by means of polyclonal antibodies. The nucleotideExpand
Cerebroside sulfatase activator deficiency induced metachromatic leukodystrophy.
Two siblings of consanguineous parents had presented with a variety of findings indicative of juvenile metachromatic leukodystrophy (MLD). However, instead of the expected profound deficiency ofExpand
Arylsulfatase B deficiency in Maroteaux-Lamy syndrome cultured fibroblasts.
Summary Fibroblasts derived from patients with Maroteaux-Lamy syndrome (mucopolysaccharidosis VI) contained about 10% of normal arylsulfatase B activity, other lysosomal enzymes being unaltered. TheExpand
Analysis of sulfatide from rat cerebellum and multiple sclerosis white matter by negative ion electrospray mass spectrometry.
The accumulation of sulfatide (sulfatogalactosyl cerebroside) and changes in the sulfatide species present have been examined in the cerebellum of day 6-32 aged rats and in multiple sclerosis (MS)Expand
Arylsulfatases A and B from human liver.
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