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Duke Orthopaedics
presents
Wheeless' Textbook of Orthopaedics

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



Original Text by Clifford R. Wheeless, III, MD.

Last updated by Data Trace Staff on Tuesday, September 13, 2011 1:22 pm