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Predicate | Object |
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rdf:type | |
lifeskim:mentions | |
pubmed:issue |
1
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pubmed:dateCreated |
1983-8-11
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pubmed:abstractText |
Multiple sulphatase deficiency (MSD) and metachromatic leukodystrophy (MLD) are both characterized by a deficiency of arylsulphatase A (ARS A) activity, although they are inherited as separate autosomal recessive traits. However, it has been found that the immunologically active substance with anti-ARS A antibody is present in quite normal levels in MLD and in smaller quantities in MSD fibroblasts (Fiddler, 1979). Indirect immunofluorescence staining with anti-ARS A antibody displayed a coarse granular and diffuse distribution of ARS A or cross-reacting material (CRM) in the normal control and MLD fibroblasts, whereas very weak fluorescence staining was observed in MSD fibroblasts proportional to the decrease in the ARS A activity observed in the lysate enzyme assay. These results suggest that ARS A deficiency in MLD cells is due to an enzymatically deficient ARS A molecule, which is immunologically cross-reactive with anti-normal ARS A antibody. ARS A deficiency in MSD cells appears to be due to a reduced amount of normal ARS A.
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pubmed:language |
eng
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pubmed:journal | |
pubmed:citationSubset |
IM
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pubmed:chemical | |
pubmed:status |
MEDLINE
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pubmed:issn |
0141-8955
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pubmed:author | |
pubmed:issnType |
Print
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pubmed:volume |
6
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pubmed:owner |
NLM
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pubmed:authorsComplete |
Y
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pubmed:pagination |
21-6
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pubmed:dateRevised |
2007-3-21
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pubmed:meshHeading |
pubmed-meshheading:6134864-Antibodies,
pubmed-meshheading:6134864-Arylsulfatases,
pubmed-meshheading:6134864-Cells, Cultured,
pubmed-meshheading:6134864-Cerebroside-Sulfatase,
pubmed-meshheading:6134864-Chromosome Aberrations,
pubmed-meshheading:6134864-Chromosome Disorders,
pubmed-meshheading:6134864-Fibroblasts,
pubmed-meshheading:6134864-Fluorescent Antibody Technique,
pubmed-meshheading:6134864-Humans,
pubmed-meshheading:6134864-Immunodiffusion,
pubmed-meshheading:6134864-Leukodystrophy, Metachromatic,
pubmed-meshheading:6134864-Skin,
pubmed-meshheading:6134864-Sulfatases
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pubmed:year |
1983
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pubmed:articleTitle |
Immunofluorescence staining and immunological studies of arylsulphatase A of multiple sulphatase deficiency (MSD) and metachromatic leukodystrophy (MLD) fibroblasts.
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pubmed:publicationType |
Journal Article,
Research Support, Non-U.S. Gov't
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