青霉胺 (Cuprimine, Depen)

概览

Penicillamine is a chelator used to treat Wilson's disease, cystinuria, and rheumatoid arthritis.

作用机制

Penicillamine is a chelating agent recommended for the removal of excess copper in patients with Wilson's disease. From in vitro studies which indicate that one atom of copper combines with two molecules of penicillamine. Penicillamine also reduces excess cystine excretion in cystinuria. This is done, at least in part, by disulfide interchange between penicillamine and cystine, resulting in formation of penicillamine-cysteine disulfide, a substance that is much more soluble than cystine and is excreted readily. Penicillamine interferes with the formation of cross-links between tropocollagen molecules and cleaves them when newly formed. The mechanism of action of penicillamine in rheumatoid arthritis is unknown although it appears to suppress disease activity. Unlike cytotoxic immunosuppressants, penicillamine markedly lowers IgM rheumatoid factor but produces no significant depression in absolute levels of serum immunoglobulins. Also unlike cytotoxic immunosuppressants which act on both, penicillamine in vitro depresses T-cell activity but not B-cell activity.

适应症

For treatment of Wilson's disease, cystinuria and active rheumatoid arthritis.

• Cystinuria
• Lead Poisoning
• Wilson's Disease
• Refractory Rheumatoid arthritis
• Severe Rheumatoid arthritis