体外ファブリとポンペ病の薬理学的シャペロン応答性をテストする酵素測定

0:35

Site-directed Mutagenesis

1:45

Transformation and Screening for the Desired Clone

2:35

pcDNA3.1/GLA and pcDNA3.1/GAA Plasmid Transfection and Treatment of HEK293H

4:00

Cell Harvest

4:43

Homogenization by Freezing and Thawing and BCA Assay

6:17

Enzyme Activity Measurement with Artificial 4-Methylumbeliferyl Substrate

8:19

Results: Enzyme Activity Measurement Mutagenized 3 α-galactosidase and 3 Acid α-glucosidase

9:35

Conclusion