Phenotypic and genetic characteristics of 130 patients with mucopolysaccharidosis type II: a single-center retrospective study in China. Front Genet. 2023;14. https://doi.org/10.3389/fgene.2023.1103620. Zhang W, et al. Genetic analysis of 63 Chinese patients with mucopolysaccharidosis type II...
Thermogravimetric analysis and discoloration tests were used to characterize the thermal stabilities. Dynamic stability analysis was used to test the processability of formulations. Compared with DOTP and DINP plasticized samples, CGE/PVC has a maximum decrease of 9.27% in glass transition temperature ( ...
(D) GST- PARG1 and GST protein were incubated with commercial PAR, and then subjected to immune-dot blot analysis by anti-PAR antibody. assay. The mutation completely destroyed the PAR hydrolase activity (Fig. 3B). As a control, the GST tag did not cause any mobility change of the ...
TGA/DSC analysis(Fig.S3)was conducted to quantify the oxide loading on KB carbon in the Co3O4-CeO2/Co-N-C catalyst.The composites were heated from room temperature to 900°C in air with a ramp rate of 10°C min−1.A slight mass loss below 100°C is due to the release of the...
Using a microplate reader, absorbance was measured at a wavelength of 570 nm. Western blot analysis. The total proteins of the UMUC-3 and T24 cells were extracted with ice-cold RIPA buffer combined with protease and phosphatase inhibitor cocktail (Cowin Biotech Co., Ltd., Beijing, China...
as a result of GAG storage, the proper functioning of the lysosome is disturbed; then, the cells, and finally, tissue, organs, and the whole organism malfunctions are observed. Due to the rarity, heterogeneity, and multi-systemic and progressive nature of MPS, they present a major diagnostic...
Fig. 1-a shows photographs of AGE and CGE in a glass bottle. The GPEs remained in a quasi-solid state at room temperature and they did not drop for a long time, even when in the horizontal state shown. The gel electrolytes were easily shapeable, translucent, and in a non-liquid state...
SDS-CGE had higher resolution, but required a long analysis and had low peak efficiency. CZE could successfully separate both PEG-5K- and PEG-20K-conjugated G-CSFs with a running time of 20 min and high peak efficiency. In conclusion, CZE was better than SDS-CGE for separating PEG-G-...
doi:10.1111/cge.12507lysosomal storage disordersmucopolysaccharidosis type IScheie syndromesignal peptide mutationMucopolysaccharidosis type I is a rare autosomal recessive disorder caused by deficiency of -l-iduronidase (IDUA) which leads to a wide spectrum of clinical severity. Here, we describe ...
Thermal properties, degradation properties and compatibility of the PVC films were investigated by Differential scanning calorimeter analysis (DSC), Thermogravimetric analysis (TGA) and FTIR, respectively. Compared with the commercial plasticizers dioctylphthalate (DOP), CGE can endow PVC film with a ...