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Fig. 2. (A) Immunoblot analysis of MPS IIIB NSC lysates. The <t>NAGLU</t> antibody identified a band in control but not in MPS IIIB lysates. (B) NAGLU activity in cell lysates from patient-derived NSCs. A significant reduced activity was observed in MPS IIIB NSCs. (C) Quantitative GAG assays to measure overall elevation of GAG in MPS IIIB NSCs. (D), (E) Representative images and quantitative analysis of Lysotracker® staining in iPSC derived NSCs. (F), (G) Representative images and quan titative analysis of Nile red staining in iPSC derived NSCs.
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Fig. 2. (A) Immunoblot analysis of MPS IIIB NSC lysates. The <t>NAGLU</t> antibody identified a band in control but not in MPS IIIB lysates. (B) NAGLU activity in cell lysates from patient-derived NSCs. A significant reduced activity was observed in MPS IIIB NSCs. (C) Quantitative GAG assays to measure overall elevation of GAG in MPS IIIB NSCs. (D), (E) Representative images and quantitative analysis of Lysotracker® staining in iPSC derived NSCs. (F), (G) Representative images and quan titative analysis of Nile red staining in iPSC derived NSCs.
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Fig. 2. (A) Immunoblot analysis of MPS IIIB NSC lysates. The <t>NAGLU</t> antibody identified a band in control but not in MPS IIIB lysates. (B) NAGLU activity in cell lysates from patient-derived NSCs. A significant reduced activity was observed in MPS IIIB NSCs. (C) Quantitative GAG assays to measure overall elevation of GAG in MPS IIIB NSCs. (D), (E) Representative images and quantitative analysis of Lysotracker® staining in iPSC derived NSCs. (F), (G) Representative images and quan titative analysis of Nile red staining in iPSC derived NSCs.
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Fig. 2. (A) Immunoblot analysis of MPS IIIB NSC lysates. The <t>NAGLU</t> antibody identified a band in control but not in MPS IIIB lysates. (B) NAGLU activity in cell lysates from patient-derived NSCs. A significant reduced activity was observed in MPS IIIB NSCs. (C) Quantitative GAG assays to measure overall elevation of GAG in MPS IIIB NSCs. (D), (E) Representative images and quantitative analysis of Lysotracker® staining in iPSC derived NSCs. (F), (G) Representative images and quan titative analysis of Nile red staining in iPSC derived NSCs.
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Fig. 2. (A) Immunoblot analysis of MPS IIIB NSC lysates. The <t>NAGLU</t> antibody identified a band in control but not in MPS IIIB lysates. (B) NAGLU activity in cell lysates from patient-derived NSCs. A significant reduced activity was observed in MPS IIIB NSCs. (C) Quantitative GAG assays to measure overall elevation of GAG in MPS IIIB NSCs. (D), (E) Representative images and quantitative analysis of Lysotracker® staining in iPSC derived NSCs. (F), (G) Representative images and quan titative analysis of Nile red staining in iPSC derived NSCs.
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Fig. 2. (A) Immunoblot analysis of MPS IIIB NSC lysates. The <t>NAGLU</t> antibody identified a band in control but not in MPS IIIB lysates. (B) NAGLU activity in cell lysates from patient-derived NSCs. A significant reduced activity was observed in MPS IIIB NSCs. (C) Quantitative GAG assays to measure overall elevation of GAG in MPS IIIB NSCs. (D), (E) Representative images and quantitative analysis of Lysotracker® staining in iPSC derived NSCs. (F), (G) Representative images and quan titative analysis of Nile red staining in iPSC derived NSCs.
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Fig. 2. (A) Immunoblot analysis of MPS IIIB NSC lysates. The NAGLU antibody identified a band in control but not in MPS IIIB lysates. (B) NAGLU activity in cell lysates from patient-derived NSCs. A significant reduced activity was observed in MPS IIIB NSCs. (C) Quantitative GAG assays to measure overall elevation of GAG in MPS IIIB NSCs. (D), (E) Representative images and quantitative analysis of Lysotracker® staining in iPSC derived NSCs. (F), (G) Representative images and quan titative analysis of Nile red staining in iPSC derived NSCs.

Journal: Experimental cell research

Article Title: Disease modeling for Mucopolysaccharidosis type IIIB using patient derived induced pluripotent stem cells.

doi: 10.1016/j.yexcr.2021.112785

Figure Lengend Snippet: Fig. 2. (A) Immunoblot analysis of MPS IIIB NSC lysates. The NAGLU antibody identified a band in control but not in MPS IIIB lysates. (B) NAGLU activity in cell lysates from patient-derived NSCs. A significant reduced activity was observed in MPS IIIB NSCs. (C) Quantitative GAG assays to measure overall elevation of GAG in MPS IIIB NSCs. (D), (E) Representative images and quantitative analysis of Lysotracker® staining in iPSC derived NSCs. (F), (G) Representative images and quan titative analysis of Nile red staining in iPSC derived NSCs.

Article Snippet: Human recombinant NAGLU (rhNAGLU) was purchased from R&D system (MN, USA), >95% purity.

Techniques: Western Blot, Control, Activity Assay, Derivative Assay, Staining

Fig. 3. Effects of rhNAGLU and δ-tocopherol (DT) in MPS IIIB NSCs. (A), (B) Representative images and quantitative analysis of LysoTracker® staining in MPS IIIB NSCs after treated with rhNAGLU. (C), (D) Representative images and quantitative analysis of LysoTracker® staining in MPS IIIB NSCs after treated with DT. (E), (F) Representative images and quantitative analysis of Nile red staining in MPS IIIB NSCs after treated with DT.

Journal: Experimental cell research

Article Title: Disease modeling for Mucopolysaccharidosis type IIIB using patient derived induced pluripotent stem cells.

doi: 10.1016/j.yexcr.2021.112785

Figure Lengend Snippet: Fig. 3. Effects of rhNAGLU and δ-tocopherol (DT) in MPS IIIB NSCs. (A), (B) Representative images and quantitative analysis of LysoTracker® staining in MPS IIIB NSCs after treated with rhNAGLU. (C), (D) Representative images and quantitative analysis of LysoTracker® staining in MPS IIIB NSCs after treated with DT. (E), (F) Representative images and quantitative analysis of Nile red staining in MPS IIIB NSCs after treated with DT.

Article Snippet: Human recombinant NAGLU (rhNAGLU) was purchased from R&D system (MN, USA), >95% purity.

Techniques: Staining