Collagen dysregulation in the dermis of the Sagg/+ mouse: a loose skin model.
Collagen dysregulation in the dermis of the Sagg/+ mouse: a loose skin model.
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Sagg/小鼠真皮中的胶原蛋白失调：松弛的皮肤模型。
DOI：
10.1038/sj.jid.5700100
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发表时间：
2006
期刊：
影响因子：
--
通讯作者：
Birk,DavidE
中科院分区：
文献类型：
--
作者：
Christner,PaulJ;Gentiletti,Julieta;Peters,Josephine;Ball,SimonT;Yamauchi,Mitsuo;Atsawasuwan,Phimon;Beason,DavidP;Soslowsky,LouisJ;Birk,DavidE
agg/+小鼠是一种乙基亚基源衍生的突变体，其皮肤表型与ehers - danlos综合征（EDS）和皮肤松弛症的某些亚型相似。与对照组相比，theSagg/+小鼠真皮的胶原纤维密度更低，结构更混乱。提取的I型真皮胶原蛋白大小与正常皮肤相同；然而，从msagg /+皮肤中可以提取更多的胶原，胶原含量降低，α1(I)、α2(I)、α1(V)和α2(V)前胶原mrna的稳态水平降低。与正常皮肤相比，sagg /+皮肤的生物力学性能明显降低。然而，在主要胶原交联蛋白的数量上，即脱氢羟基赖氨酸氨基亮氨酸和脱氢组氨酸二羟氨基葡萄糖的数量在皮肤与正常皮肤之间没有显著差异。对sagg /+皮肤的电镜评价表明，该突变干扰了胶原原纤维的正常形成，这一数据与V型胶原的突变导致单倍功能不全一致，形成了两个胶原原纤维亚群，一个正常，一个形状不规则，直径较大。对这种新突变的进一步研究将允许识别参与正常和病理胶原基因表达调节的新机制。
TheSagg/+ 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 theSagg/+ 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 fromSagg/+ skin, which also showed decreased collagen content and decreased steady-state levels ofα1(I),α2(I),α1(V), andα2(V) procollagen mRNAs. The biomechanical properties ofSagg/+ 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 betweenSagg/+ and normal skin. Electron microscopic evaluation ofSagg/+ 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.
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DOI：
10.1007/3-540-29662-x_936
发表时间：
2022
期刊：
Neurocutaneous Disorders
影响因子：
--
作者：
Larry W. Moreland
通讯作者：
Larry W. Moreland
皮肤胶原蛋白中稳定的组氨酸三官能交联的结构和形成。
DOI：
--
发表时间：
1987
期刊：
The Journal of biological chemistry
影响因子：
--
作者：
Yamauchi,M;London,RE;Guenat,C;Hashimoto,F;Mechanic,GL
通讯作者：
Mechanic,GL
影响因子：
4
作者：
D. Birk;J. Fitch;J. Babiarz;K. Doane;T. Linsenmayer
通讯作者：
D. Birk;J. Fitch;J. Babiarz;K. Doane;T. Linsenmayer
影响因子：
4.2
作者：
L. Soslowsky;C. H. An;S. P. Johnston;J. Carpenter
通讯作者：
L. Soslowsky;C. H. An;S. P. Johnston;J. Carpenter
与先天性皮肤松弛相关的神经轴索脑白质营养不良：尸检病例报告
影响因子：
12.7
作者：
M. Shintaku;Y. Uemura;I. Fujii;Y. Ohtani;T. Miike;M. Tokunaga;A. Tsubura
通讯作者：
A. Tsubura