Collagen dysregulation in the dermis of the Sagg/+ mouse: a loose skin model

摘要: The Sagg /+ mouse is an ethylnitrosourea-derived mutant with a dermal phenotype similar to some of the subtypes Ehlers–Danlos syndrome (EDS) and cutis laxa. dermis has less dense more disorganized collagen fibers compared controls. size extracted Type I was same as that observed in normal skin; however, could be from skin, which also showed decreased content steady-state levels α 1(I), 2(I), 1(V), 2(V) procollagen mRNAs. biomechanical properties skin were significantly relative skin. However, there no significant differences quantities major cross-links, is, dehydrohydroxylysinonorleucine dehydrohistidinohydroxymerodesmosine between Electron microscopic evaluation indicated mutation interferes proper formation fibrils data are consistent V leading haploinsufficiency two sub-populations fibrils, one irregular shape larger diameter. Further study this novel will allow identification new mechanisms involved regulation pathologic gene expression.