| Autor: |
Rowe, David W., McGoodwin, Ermona B., Martin, George R., Sussman, Michael D., Grahn, Douglas, Faris, Barbara, Franzblau, Carl |
| Zdroj: |
The Journal of Experimental Medicine; January 1974, Vol. 139 Issue: 1 p180-192, 13p |
| Abstrakt: |
A genetic abnormality in collagen and elastin cross-linking resembling experimental lathyrism has been identified in mice. The defect is an X-linked trait, attributed to the mottled locus which also influences coat color. The affected mice have aneurysms of the aorta and its branches, weak skin, and bone deformities in a spectrum of severity varying with the alleles at the mottled locus. A defect in the cross-linking of collagen was demonstrated in the skin of the affected animals by a marked increase in collagen extractability and a reduced proportion of cross-linked components in the extracted collagen. A decrease in lysine-derived aldehyde levels was found in both skin collagen and aortic elastin similar to that found in lathyritic tissue. Furthermore the in vitro formation of lysine-derived aldehyde was reduced. Thus the cause of the connective tissue abnormalities in these mice appears to be a defect in cross-link formation due to an impairment in aldehyde formation. |
| Databáze: |
Supplemental Index |
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