Additional file 1 of Neural stem cells for disease modeling and evaluation of therapeutics for infantile (CLN1/PPT1) and late infantile (CLN2/TPP1) neuronal ceroid lipofuscinoses

Abstract

Figure S1. Generation of Neuronal Ceroid Lipofuscinosis (NCL) induced pluripotent stem cells (iPSCs). Figure S2. Pluripotent stem cell protein markers analyzed by flow cytometry. Figure S3. Immunofluorescence staining of iPSC pluripotent stem cell protein markers. Figure S4. Protein marker expression in NSCs differentiated from WT control and NCL patient iPSCs. Figure S5. Enlarged lysosomes and lipid accumulation in NCL patient fibroblasts. Figure S6. Filipin staining in NCL fibroblasts and NSCs. Figure S7. Cytotoxicity of DT and HPBCD on NCL patient NSCs. Figure S8. Lysosomal pH indicated by a pHrodo™ pH sensor dye in NCL patient NSCs. (PDF 1842 kb