Figure 1.

Long-Term Treatment of the I278T Mice with PEG-CBS Results in a Sustained Improvement in Plasma Sulfur Amino Acid Profiles

Plasma concentrations of Hcy (solid line, squares), Cth (dashed line, circles), and Cys (dotted line, diamonds) are corrected in asymptomatic 21-day-old mice by three weekly s.c. injections of 7.5 mg/kg PEG-CBS (n = 3; A). The improvement is sustained over the subsequent ∼9-month treatment period. A similar, sustained improvement is observed in symptomatic 26-week-old mice (n = 10; B) treated with PEG-CBS for ∼6 months. For each metabolite, the initial data point represents the concentration prior to treatment, while subsequent data points represent concentrations of plasma metabolites 24 hr post-injection.