Collagen Defect induced by Penicillamine

ALDEHYDES derived from lysine are involved in the crosslinking of collagen and elastin1-3 and abnormalities in crosslinking can be induced by lathyrogens such as β-amino-propionitrile, isonicotinic acid hydrazide and semicarbazide4,5 as well as by D-penicillamine6,7. Lathyrogens seem to inhibit aldehyde synthesis by irreversibly inactivating a lysyl oxidase in connective tissue8. Although penicillamine at the usual dosage does not affect the activity of this enzyme, it seems to prevent crosslinking by binding to the peptide-bound aldehydes on collagen9.