penicillamine-disulfide and Hepatolenticular-Degeneration

Penicillamine disulfides have been analysed by automatic amino acid analysis. Because the free thiol reacts poorly with ninhydrin, other detection methods are preferred, particularly high pressure liquid chromatography using an electrochemical detector or gas chromatography with a flame ionization detector. Pharmacokinetic studies have now been reported using these techniques. With patients on established penicillamine regimes, the concentration of free penicillamine in the plasma has been found to vary between 4 and 20 microM depending on dosage and time of administration. Disulfide concentration is higher than this by a factor of 3 or 4 and an even greater quantity is attached to plasma and tissue proteins.

Topics: Arthritis, Rheumatoid; Chromatography, Gas; Chromatography, High Pressure Liquid; Cystinuria; Dose-Response Relationship, Drug; Hepatolenticular Degeneration; Humans; Penicillamine; Protein Binding