Desmosine Biosynthesis: Nature of Inhibition by D-Penicillamine

Administration of D-penicillamine and lathyrogens such as β-amino-propionitrile to animals markedly alters connective tissue by preventing the normal cross-linkage of elastin and collagen. It had been shown that β-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 α-amino adipic-δ-semialdehyde. We show that penicillamine acts after the initial step, causing the accumulation of an elastin rich in α-amino adipic-δ-semialdehyde.