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- W4384207856 abstract "Mucopolysaccharidosis Type I (MPSI) is a rare inherited lysosomal storage disease that arises due to mutations in the IDUA gene. Defective alpha-L-iduronidase (IDUA) enzyme is unable to break down glucosaminoglycans (GAGs) within the lysosomes and, as a result, there is systemic accumulation of undegraded products in lysosomes throughout the body leading to multi-system disease. Here, we characterised the skeletal/craniofacial, neuromuscular and behavioural outcomes of the MPSI Idua-W392X mouse model. We demonstrate that Idua-W392X mice have gross craniofacial abnormalities, showed signs of kyphosis, and show signs of hypoactivity compared to wild-type mice. X-ray imaging analysis revealed significantly shorter and wider tibias and femurs, significantly wider snouts, increased skull width and significantly thicker zygomatic arch bones in Idua-W392X female mice compared to wild-type mice at 9 and 10.5 months of age. Idua-W392X mice display decreased muscle strength, especially in the forelimbs, which is already apparent from 3 months of age. Female Idua-W392X mice display hypoactivity in the open-field test from 9 months of age and anxiety-like behaviour at 10 months of age. As these behaviours have been identified in Hurler children, the MPSI Idua-W392X mouse model may be important for the investigation of new therapeutic approaches for MPSI-Hurler." @default.
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- W4384207856 date "2023-08-01" @default.
- W4384207856 modified "2023-09-26" @default.
- W4384207856 title "Phenotypic characterisation of the Mucopolysaccharidosis Type I (MPSI) Idua-W392X mouse model reveals increased anxiety-related traits in female mice" @default.
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- W4384207856 doi "https://doi.org/10.1016/j.ymgme.2023.107651" @default.
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