Characterisation of the T cell and dendritic cell repertoire in a murine model of mucopolysaccharidosis I (MPS I)
Journal of inherited metabolic disease | 10 Jul 2012
LD Archer, KJ Langford-Smith, WR Critchley, BW Bigger and JE Fildes
Mucopolysaccharidosis I (MPS I) is a metabolic disorder caused by α-L-Iduronidase (IDUA) deficiency, resulting in lysosomal accumulation of heparan (HS) and dermatan sulphate (DS). This has been reported in microglia, yet currently the effect of IDUA deficiency on T cells and dendritic cells (DC) and their functionality in disease pathogenesis remains unclear.
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