Lathyrism

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- 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;