Heterogeneity in dermatosparaxis is shown by contraction of collagen gels.
Dermal fibroblasts from sheep exhibiting a mild form of dermatosparaxis were able to contract reconstituted, fibrillar collagen gels at the same rate as control dermal fibroblasts, indicating a normal interaction between the cells and a collagenous matrix. An extract from dermatosparactic skin was shown, after partial purification, to have N-proteinase activity, although the level of activity was much lower than found in normal skin. These data show that dermatosparaxis is a heterogeneous disease, since in the severe forms of the disease the defect has been characterized as an absence of N-proteinase and an inability of the cells to interact with and contract collagen gels.
Ramshaw
J A
JA
CSIRO, Division of Biomolecular Engineering, Parkville, Victoria, Australia.
Mitrangas
K
K
Bateman
J F
JF
D000818:Animals; D002352:Carrier Proteins / Q000378:metabolism; D002448:Cell Adhesion; D002460:Cell Line; D003094:Collagen / Q000378:metabolism*; D003240:Connective Tissue Diseases / Q000378:metabolism / Q000473:pathology / Q000662:veterinary*; D005347:Fibroblasts / Q000502:physiology*; D005782:Gels; D011348:Procollagen N-Endopeptidase / Q000172:deficiency / Q000378:metabolism; D012756:Sheep
D002352:Carrier Proteins; D005782:Gels; D003094:Collagen; D011348:Procollagen N-Endopeptidase