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Fig. 4 | Orphanet Journal of Rare Diseases

Fig. 4

From: Effect of systemic high dose enzyme replacement therapy on the improvement of CNS defects in a mouse model of mucopolysaccharidosis type II

Fig. 4

Histology of brain tissues such as the cerebral cortex, thalamus, and cerebellum with anti-Lamp-2 and H&E (200X). In group A, the untreated KO mice showed increased anti-Lamp-2 immunostaining compared with the WT mice. No treatment group showed reduced anti-Lamp-2 immunostaining (a). In group B, Lamp-2 immunostaining was evident in all areas of the untreated KO mice. A definite reduction in Lamp-2 immunostaining was observed in all treated groups at 5 mg/kg, 5 mg/kg plus mannitol pretreatment, or 10 mg/kg. The two dose-change groups did not reveal remarkable change in Lamp-2 immunostaining (b). In group C, the area of Lamp-2 immunostaining was less massive in the untreated MPS II mice compared with that of the untreated KO mice of group B. There was no significant reduction of Lamp-2 immunostaining in any treatment group (c). The number of distinct and enhanced neuronal vacuolation (arrow) in perivascular cells and neurons was markedly increased in the untreated KO groups of groups A, B, and C. Dyslamination and decreased cellular density were noted in the cortical area of the untreated KO groups. In group A, the reduced vacuolation was not remarkable in all treatment groups, even in the KO mice treated with 10 mg/kg after 4 weeks of ERT (d). In group B, the degree of vacuolation was more severe than in the untreated KO mice of group A. After 24 weeks of high-dose ERT (5 mg/kg/week or higher), the brain tissues of the KO mice showed a reduced degree of vacuolation, which was not remarkable in the two dose-change groups (e). In group C, the brain tissues of the treated KO mice did not show reduced vacuolation after 12 weeks of ERT (f)

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