Frey J, Chamson A, Gourreau J M, Gillet J P
Laboratoire de Biochimie, Faculté de Médecine, Saint Etienne, France.
J Invest Dermatol. 1989 Jul;93(1):83-6. doi: 10.1111/1523-1747.ep12277360.
An analysis of the dermis and a study of collagen and lipid biosynthesis by fibroblast cultures were carried out in one case of epitheliogenesis imperfecta in a new born calf. This was found to be not only an epidermal disease with fissures and blisters within basal cells on the basement membrane but also a metabolic disease affecting the dermal fibroblasts. These fibroblasts showed a significant decrease in collagen biosynthesis with an increase in the percentage of type III collagen and also a decrease in the biosynthesis of lipids, especially glycerides and cholesterol.
对一头新生小牛的一例上皮形成不全病例进行了真皮分析以及成纤维细胞培养物中胶原蛋白和脂质生物合成的研究。结果发现,这不仅是一种在基底膜上的基底细胞内出现裂隙和水疱的表皮疾病,而且是一种影响真皮成纤维细胞的代谢疾病。这些成纤维细胞显示胶原蛋白生物合成显著减少,III型胶原蛋白百分比增加,脂质尤其是甘油酯和胆固醇的生物合成也减少。