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Hoffmann, R. A wiki for the life sciences where authorship matters. Nature Genetics (2008)
 
 
 

Elastic fibers of musculoskeletal tissues in bovine Marfan syndrome: a morphometric study.

Bovine Marfan syndrome has all the major pathognomonic, clinical, and pathological features of the human syndrome. To further explore the alterations characterizing Marfan syndrome, musculoskeletal tissues from affected and healthy cattle were subjected to histochemical and histomorphometric analysis. Our findings demonstrated reduced elastic fiber content in the periosteum, capsule, interosseous ligament, and flexor tendons of the metatarsophalangeal joint of affected cattle. The elastic properties of a tissue are supplied by elastic fibers in the extracellular matrix; therefore, their reduced content in articular tissues suggests that these fibers could be functionally incompetent to withstand normal stress, causing a predisposition to joint laxity and dislocation. Moreover, perichondrial-periosteal membranes, which are made of collagen and elastic fibers, are known to affect the growth process of the long bones. The decrease in the number of elastic fibers in these membranes could result in reduced restraint of skeletal growth and explain some skeletal abnormalities of Marfan syndrome (i.e., dolichostenomelic habitus).[1]

References

  1. Elastic fibers of musculoskeletal tissues in bovine Marfan syndrome: a morphometric study. Gigante, A., Chillemi, C., Potter, K.A., Bertoni-Freddari, C., Greco, F. J. Orthop. Res. (1999) [Pubmed]
 
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