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Dysregulation of the lysosome function in MPS I. (A) Expression of genes from KEGG lysosome pathway (hsa04142). Expression level is log2 CPM. (B) Validation of lysosome genes by qRT-PCR. (C and D) Immunoblot validation of selected lysosome genes. (E) Immunohistochemical analysis of lysosome membrane proteins in the NSCs from the MPS I isotypes.
