Additional file 1 of Ceria-Zirconia nanoparticles reduce intracellular globotriaosylceramide accumulation and attenuate kidney injury by enhancing the autophagy flux in cellular and animal models of Fabry disease
Creators: name: An, Jong Hun
name: Hong, Sang-Eun
name: Yu, Seong-Lan
name: Kang, Jaeku
name: Park, Chang Gyo
name: Lee, Hoi Young
name: Lee, Sung-Ki
name: Lee, Dong Chul
name: Park, Hwan-Woo
name: Hwang, Won-Min
name: Yun, Sung-Ro
name: Park, Yohan
name: Park, Moon Hyang
name: Yoon, Kuk Ro
name: Yoon, Se-Hee
Publisher: name: figshare
Year: 2022
Subjects: subject: Medicine
subject: Cell Biology
subject: Molecular Biology
subject: Neuroscience
subject: Physiology
subject: FOS: Biological sciences
subject: 39999 Chemical Sciences not elsewhere classified
subject: FOS: Chemical sciences
subject: Science Policy
subject: Immunology
subject: FOS: Clinical medicine
subject: Marine Biology
subject: Cancer
subject: Hematology
subject: 110309 Infectious Diseases
subject: FOS: Health sciences

description: Additional file 1: Fig S1. Schematic representation of synthesized PEG-CZNPs and FITC conjugated PEG-CZNPs. A The process comprises two steps; (i) preparation of CZNPs based on non-hydrolytic sol-gel reaction in oleylamine; (ii) transfer of the as-synthesized CZNPs to aqueous phase by modifying with phospholipid-PEG. B FITC-conjugated phospholipid-PEG capped CZNPs were prepared with a thiourea linkage. C Analysis of Fourier transform infrared spectroscopy (FT-IR) spectra comparing the as synthesized PEG-CZNP and FITC conjugated PEG-CZNP with FITC. (a) PEG-CZNP, (b) FITC and (c) FITC conjugated PEG-CZNP. Fig. S2. Expression of key differentiation markers in human podocytes. A, B Representative confocal immunofluorescence microscopy images of podocin, nephrin and synaptopodin (green), with DAPI counterstaining (blue), in both undifferentiated and differentiated human podocytes. Fig. S3. Validation of α-GLA knockdown efficiency by quantitative real-time RT-PCR analysis and immunoblotting. A, B Quantitative real-time RT-PCR and immunoblotting results showing the α-GLA knockdown efficiency in HK-2 cells and human podocytes. Data values represent the mean ± SD; *P&lt;0.05 and **P&lt;0.01 versus control. Fig. S4. Effects of PEG-CZNPs on cell viability. The viability of HK-2 cells (A) and matured human podocytes (B) treated with PEG-CZNP was measured using the MTT assay. Data values represent the mean ± SD; *P&lt;0.05 and ***P&lt;0.001 versus 0 μg/mL. Fig. S5. Schema for the PET-CZNP treatments of the mouse model of FD. PEG-CZNPs (10 mg/kg) or normal saline (2 mL/kg) were administered intraperitoneally to the mice twice per week from 4 to 12 weeks of age. The mice were sacrificed at 12 weeks of age. Fig. S6. Intracellular localization and biodistribution of the PEG-CZNPs. Confocal microscopy analysis of human podocyte treated with FITC-labeled PEG-CZNPs (green) after 0, 4, 8, 16, 24, 48, 72 and 96 hr. The human podocytes were then stained with Lysotracker (red). Fig. S7. Biodistribution analysis of PEG-CZNPs. ICP-MS analysis of the ceria (A) and zirconia (B) contents in the organs of B6 mice after the intraperitoneal injection of 10mg/kg PEG-CZNPs twice a week for 12 weeks. Fig. S8. ELISA measurements of the GLA protein levels in plasma samples from the mouse model of FD. Group1; wild type, normal saline injection, Group 2; wild type + PEG-CZNP injection, Group 3; B6;129-Glatm1Kul/J mice + normal saline injection and Group 4; B6;129-Glatm1Kul/J mice + PEG-CZNP injection. Data values represent the mean ± SD; *P&lt;0.05, ***P &lt; 0.001 versus Group 1.