Article
Murine mucopolysaccharidosis type I: targeted disruption of the murine alpha-L-iduronidase gene.
Human molecular genetics - 1 Apr 1997
Clarke L A, Russell C S, Pownall S, Warrington C L, Borowski A, Dimmick J E, Toone J, Jirik F R
Abstract excerpt
Mucopolysaccharidosis type I (MPS I) is considered to represent the prototypical mucopolysaccharide storage disorder. Although a spectrum of severity is seen within the MPS I subgroup, Hurler syndrome represents the most severe and frequent manifestation of MPS I. We describe here the generation of a murine model for Hurler syndrome by targeted disruption of the murine Idua gene. Homozygous Idua -/- mice have no...
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