Desmosine biosynthesis: nature of inhibition by D-penicillamine.

Administration of D-penicillamine and lathyrogens such as beta-amino-propionitrile to animals markedly alters connective tissue by preventing the normal cross-linkage of elastin and collagen. It had been shown that beta-aminopropionitrile blocks the cross-linkage of elastin and collagen by preventing the initial step in cross-linkage: the conversion of lysine in peptide linkage to alpha-amino adipic-delta-semialdehyde. We show that penicillamine acts after the initial step, causing the accumulation of an elastin rich in alpha-amino adipic-delta-semialdehyde.