ABNORMALITIES OF COLLAGEN FIBRILS IN A RABBIT WITH A CONNECTIVE-TISSUE DEFECT SIMILAR TO EHLERS-DANLOS SYNDROME

A morphometric ultrastructural study was performed to confirm the presence of an abnormality of the collagen fibrils in a rabbit with a connective tissue defect similar to Ehlers-Danlos syndrome. Median fibril diameter and perimeter were not altered but their ranges were significantly increased. As indicated by the median fibril 'form factor', fibrils were significantly more irregular in shape; the range of irregularity in shape was also increased. Fibril periodicity was unchanged. The results are discussed in relation to collagen fibril structure and fibril abnormalities in similar diseases in man and other animals.