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Lathyrism



- 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