| First Author | Christner PJ | Year | 2006 |
| Journal | J Invest Dermatol | Volume | 126 |
| Issue | 3 | Pages | 595-602 |
| PubMed ID | 16424879 | Mgi Jnum | J:105704 |
| Mgi Id | MGI:3616360 | Doi | 10.1038/sj.jid.5700100 |
| Citation | Christner PJ, et al. (2006) Collagen dysregulation in the dermis of the sagg/+ mouse: a loose skin model. J Invest Dermatol 126(3):595-602 |
| abstractText | The Sagg/+ mouse is an ethylnitrosourea-derived mutant with a dermal phenotype similar to some of the subtypes of Ehlers-Danlos syndrome (EDS) and cutis laxa. The dermis of the Sagg/+ mouse has less dense and more disorganized collagen fibers compared to controls. The size of extracted Type I dermal collagen was the same as that observed in normal skin; however, more collagen could be extracted from Sagg/+ skin, which also showed decreased collagen content and decreased steady-state levels of alpha1(I), alpha2(I), alpha1(V), and alpha2(V) procollagen mRNAs. The biomechanical properties of Sagg/+ skin were significantly decreased relative to normal skin. However, there were no significant differences in the quantities of the major collagen cross-links, that is, dehydrohydroxylysinonorleucine and dehydrohistidinohydroxymerodesmosine between Sagg/+ and normal skin. Electron microscopic evaluation of Sagg/+ skin indicated that the mutation interferes with the proper formation of collagen fibrils and the data are consistent with a mutation in Type V collagen leading to haploinsufficiency with the formation of two sub-populations of collagen fibrils, one normal and one with irregular shape and a larger diameter. Further study of this novel mutation will allow the identification of new mechanisms involved in the regulation of normal and pathologic collagen gene expression.Journal of Investigative Dermatology (2006) 126, 595-602. doi:10.1038/sj.jid.5700100; published online 19 January 2006. |
