Abstract:Mucopolysaccharidosis (MPS) III are a group of four lysosomal storage diseases with severe neurological manifestation. While the primary pathology of MPS III is the lysosomal storage of heparan sulfates, detailed mechanisms of neuropathology are unclear. Using a mouse model, we showed that a profound neuroimmune response contributes to the CNS disease progression of MPS IIIB. Immune status in MPS III patients remains unexplored. Here, we tested peripheral blood from 12 patients with MPS III, including MPS IIIA… Show more
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