Human alpha-L-iduronidase: cDNA isolation and expression.

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Immunopurification and characterization of human alpha-L-iduronidase with the use of monoclonal antibodies.

alpha-L-Iduronidase from human liver was purified by a three-step five-column procedure and by immunoaffinity chromatography with a monoclonal antibody raised against purified enzyme. Seven bands identified by staining with Coomassie Blue had molecular masses of 74, 65, 60, 49, 44, 18 and 13 kDa and were present in both preparations of the liver enzyme. However, relative to the immunopurificati...

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Long-term in vitro correction of alpha-L-iduronidase deficiency (Hurler syndrome) in human bone marrow.

Allogeneic bone marrow transplantation is the most effective treatment for Hurler syndrome but, since this therapy is not available to all patients, we have considered an alternative approach based on transfer and expression of the normal gene in autologous bone marrow. A retroviral vector carrying the full-length cDNA for alpha-L-iduronidase has been constructed and used to transduce bone marr...

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ISOLATION AND EXPRESSION OF cDNA CLONES

Receptors for the Fc region of IgG (FcyR)' are expressed in a number of hematopoietic cell types and play important roles in several immunological processes such as phagocytosis of opsonized particulate antigens (1), clearance of immune complexes (2, 3), antibody-dependent cellular cytotoxicity (4, 5), signalling the production of inflammatory mediators (6), and regulation of Ig synthesis (7, 8...

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Post- and pre-natal assessment of alpha-L-iduronidase deficiency with a radiolabelled natural substrate.

1. a-L-Iduronidase activity was assayed by incubation of iduronosyl anhydro[ l-3H]mannitol 6-sulphate with homogenates of cultured skin fibroblasts, amniotic cells and leucocytes derived from normal individuals, patients affected with GLiduronidase deficiency disorder (mucopolysaccharidosis type I: Hurler, Scheie and Hurler-Scheie compound) and parents of such patients. 2. The assay for cc-L-id...

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Human alpha-L-iduronidase. Catalytic properties and an integrated role in the lysosomal degradation of heparan sulphate.

The kinetic parameters (Km and kcat) of human liver alpha-L-iduronidase were determined with a variety of heparin-derived disaccharide and tetrasaccharide substrates. More structurally complex substrates, in which several aspects of the aglycone structure of the natural substrates heparin and heparan sulphate were maintained, were hydrolysed with catalytic efficiencies up to 255 times that obse...

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ژورنال

عنوان ژورنال: Proceedings of the National Academy of Sciences

سال: 1991

ISSN: 0027-8424,1091-6490

DOI: 10.1073/pnas.88.21.9695