Article
Glycosidase active site mutations in human alpha-L-iduronidase.
Glycobiology - 1 Sept 2001
Brooks D A, Fabrega S, Hein L K, Parkinson E J, Durand P, Yogalingam G, Matte U, Giugliani R, Dasvarma A, Eslahpazire J, Henrissat B, Mornon J P, Hopwood J J, Lehn P
Abstract excerpt
Mucopolysaccharidosis type I (MPS I; McKusick 25280) results from a deficiency in alpha-L-iduronidase activity. Using a bioinformatics approach, we have previously predicted the putative acid/base catalyst and nucleophile residues in the active site of this human lysosomal glycosidase to be Glu182 and Glu299, respectively. To obtain experimental evidence supporting these predictions, wild-type alpha-L-iduronidase...
Topics
- Amino Acid Sequence
- Animals
- Antibodies, Monoclonal
- Base Sequence
- Binding Sites
- Blotting, Western
- CHO Cells
- Cricetinae
- DNA Primers
- Epitope Mapping
- Glycoside Hydrolases
- Humans
- Iduronidase
- Mucopolysaccharidosis I