A Canine Model of Human α -L-iduronidase Deficiency
A Canine Model of Human α -L-iduronidase Deficiency
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Publisher
United States: National Academy of Sciences of the United States of America
Journal title
Language
English
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Publication information
Publisher
United States: National Academy of Sciences of the United States of America
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Scope and Contents
Contents
A disease discovered in three Plott Hound littermates was found to be associated with a profound and specific deficiency of α -L-iduronidase (mucopolysaccharide α -L-iduronohydrolase; EC 3.2.1.76) in fibroblasts and leukocytes. The pedigree was consistent with autosomal recessive inheritance. A markedly increased amount of dermatan sulfate and hepa...
Alternative Titles
Full title
A Canine Model of Human α -L-iduronidase Deficiency
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Record Identifier
TN_cdi_pnas_primary_80_19_6091
Permalink
https://devfeature-collection.sl.nsw.gov.au/record/TN_cdi_pnas_primary_80_19_6091
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ISSN
0027-8424
E-ISSN
1091-6490
DOI
10.1073/pnas.80.19.6091