- Discussion:
- lathyritic animals are known to have soft, deformed bones, many joint dislocations, and aortic aneurysms;
- if the extracellular processes of cross-link formation are inhibited, collagen will remain soluble, which is the case in a disease known as
lathyrism.
- ingestion of agents called lathyrogens, including b-aminopropionitrile, causes profound alterations in collagen of connective tissue & bone;
- most lathyrogens act by inhibiting the enzyme lysyl oxidase, which is responsible for the synthesis of aldehydes.
- these aldehydes are needed for formation of the bonds that link two chains together;
- copper deficiency also causes a disorder similar to lathyrism because lysyl oxidase is a copper-requiring enzyme;
- penicillamine & cystamine have been investigated for their ability to react w/ aldehydes in collagen & to break cross-linkages formed;
- if these intermolecular & intramolecular cross-linkages are inhibited or broken, the net effect is a marked increase in amount of
collagen that can be extracted under mild conditions from bone and other connective tissue