Increased collagen cross-linking is a signature of dystrophin-deficient muscle.
Increased collagen cross-linking is a signature of dystrophin-deficient muscle.
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DOI：
10.1002/mus.24998
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发表时间：
2016-06
期刊：
影响因子：
3.4
通讯作者：
Barton, Elisabeth R.
中科院分区：
文献类型：
--
作者：
Smith, Lucas R.;Hammers, David W.;Sweeney, H. Lee;Barton, Elisabeth R.
胶原交联是细胞外基质（ECM）成熟、转换和硬度的关键参数。我们研究了肌营养不良蛋白缺陷小鼠、犬和人骨骼肌中胶原交联的各个方面。分析DMD患者活组织检查和来自mdx小鼠的样品和金毛猎犬肌营养不良犬样品（具有适当的对照）。使用溶解度和羟脯氨酸测定评价胶原交联。通过真实的时间聚合酶链反应、免疫印迹和免疫荧光测定交联酶赖氨酰氧化酶（LOX）的表达。LOX蛋白水平在所有评估物种的营养不良肌肉中增加。营养不良小鼠和犬的交联胶原蛋白显著高于对照组，尤其是在膈肌中。肌内脂氧合酶的分布在所有样品中是不均匀的，但它的频率和强度增加营养不良的肌肉。这些发现暗示胶原交联升高是营养不良肌肉中破坏的ECM的重要组成部分，并且在小鼠、狗和人中交联升高是明显的。
Collagen cross‐linking is a key parameter in extracellular matrix (ECM) maturation, turnover, and stiffness. We examined aspects of collagen cross‐linking in dystrophin‐deficient murine, canine, and human skeletal muscle. DMD patient biopsies and samples from mdx mice and golden retriever muscular dystrophy dog samples (with appropriate controls) were analyzed. Collagen cross‐linking was evaluated using solubility and hydroxyproline assays. Expression of the cross‐linking enzyme lysyl oxidase (LOX) was determined by real‐time polymerase chain reaction, immunoblotting, and immunofluorescence. LOX protein levels are increased in dystrophic muscle from all species evaluated. Dystrophic mice and dogs had significantly higher cross‐linked collagen than controls, especially in the diaphragm. Distribution of intramuscular LOX was heterogeneous in all samples, but it increased in frequency and intensity in dystrophic muscle. These findings implicate elevated collagen cross‐linking as an important component of the disrupted ECM in dystrophic muscles, and heightened cross‐linking is evident in mouse, dog, and man. Muscle Nerve 54: 71–78, 2016
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