We developed a simple and sensitive stable-isotope dilution method for the quantification of 3-hydroxyglutaric acid (3HGA) and glutaric acid (GA) in body fluids. In our method, tertbutyldimethylsilyl derivatives of 3HGA and GA were measured with a conventional electron-impact ionization mode in gas chromatography-mass spectrometry. The control values for 3HGA in nmol/ml were 0.15 ± 0.08 (serum;...

Aggregation: Amyloid-β Destabilization Promotes Annular Protofibril Formation Wei-Ting Chen, Yi-Hung Liao, Hui-Ming Yu, Irene H. Cheng, and Yun-Ru Chen From Genomics Research Center, Academia Sinica, Institute of Brain Science, National Yang-Ming University, Taiwan International Graduate Program, Chemical Biology and Molecular Biophysics Program, Genomics Research Center, Academia Sinica, Insti...

Aldose reductase in a highly purified state is unstable. It requires the presence of thiol groups to maintain it in an active form. The enzyme apparently exists in 3 forms, only one of which is active. Tetramethylene glutaric acid (TMG) is an effective aldose reductase inhibitor. However, a relatively high level of TMG is needed to depress dulcitol synthesis in the lens incubated in a galactose...

Degeneration of specific neuronal populations and progressive nervous system dysfunction characterize neurodegenerative diseases, including Alzheimer's disease and Parkinson's disease. These findings are also reported in inherited diseases such as phenylketonuria and glutaric aciduria type I. The involvement of mitochondrial dysfunction in these diseases was reported, elicited by genetic altera...