Informação sobre produto
- IDUA
- α-L-Iduronidase
PAIRED PRODUCTS AVAILABLE:
4-MU-alpha-L-idopyranosiduronic acid 2-sulphate, Substrate, EM03201
4MU-alpha-L-idopyranosiduronic acid, Substrate, EM04841
Alpha-L-iduronidase is a glycoside hydrolase that cleaves the non-reducing, terminal alpha-L-iduronic acid residues from polysaccharides and oligosaccharides. Alpha-L-iduronidase is involved in the lysosomal degradation of glycosaminoglycans and its deficiency leads to the accumulation of dermatan sulphate and heparan sulphate in tissues, a condition known as mucopolysaccharidosis I (MPS I). Alpha-L-iduronidase activity can be detected and quantified in an assay using a fluorogenic substrate 4MU-alpha-L-idopyranosiduronic acid. Alpha-L-iduronidase is also used in a 2-step assay for Hunter syndrome (MPS II) testing in combination with 4-MU-alpha-L-idopyranosiduronic acid 2-sulphate.
This enzyme is supplied as a lyophilisate of: 25% (w/w) Iduronidase enzyme (protein), 37% (w/w) sucrose, 38% (w/w) MOPS potassium (buffer, pH 7.4). The enzyme is derived from a bacterial source, recombinantly expressed in Escherichia coli (see application notes for more details). Alpha-L-iduronidase is also available as an aqueous solution (FI179383)
Propriedades químicas
Consulta técnica sobre: 3D-FA181881 α-L-Iduronidase, recombinant, lyophilised
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