Ošt'ádal Martin, Eckhardt Adam, Herget Jan, Mikšík Ivan, Dungl Pavel, Chomiak Jiří, Frydrychová Monika, Burian Michal
Department of Orthopaedics, 1st Faculty of Medicine, University Hospital Bulovka, Charles University, Budínova 2, 180 00, Prague 8, Czech Republic,
Mol Cell Biochem. 2015 Mar;401(1-2):133-9. doi: 10.1007/s11010-014-2300-3. Epub 2014 Dec 4.
Idiopathic pes equinovarus is a congenital deformity of the foot and lower leg defined as a fixation of the foot in adduction, supination, and varus. Although the pathogenesis of clubfoot remains unclear, it has been suggested that fibroblasts and growth factors are involved. To directly analyze the protein composition of the extracellular matrix in contracted tissue of patients with clubfoot. A total of 13 infants with idiopathic clubfoot treated with the Ponseti method were included in the present study. Tissue samples were obtained from patients undergoing surgery for relapsed clubfeet. Contracted tissues were obtained from the medial aspect of the talonavicular joint. Protein was extracted after digestion and delipidation using zip-tip C18. Individual collagenous fractions were detected using a chemiluminescent assay. Amino acid analysis of tissue samples revealed a predominance of collagens, namely collagen types I, III, and VI. The high content of glycine and h-proline suggests a predominance of collagens I and III. A total of 19 extracellular matrix proteins were identified. The major result of the present study was the observation that the extracellular matrix in clubfoot is composed of an additional 16 proteins, including collagens V, VI, and XII, as well as the previously described collagen types I and III and transforming growth factor β. The characterization of the general protein composition of the extracellular matrix in various regions of clubfoot may help in understanding the pathogenesis of this anomaly and, thus, contribute to the development of more efficacious therapeutic approaches.
特发性马蹄内翻足是一种足和小腿的先天性畸形，定义为足处于内收、旋后和内翻位固定。尽管马蹄内翻足的发病机制尚不清楚，但有人提出成纤维细胞和生长因子与之有关。为了直接分析马蹄内翻足患者挛缩组织中细胞外基质的蛋白质组成。本研究共纳入13例接受Ponseti方法治疗的特发性马蹄内翻足婴儿。组织样本取自复发性马蹄内翻足手术患者。挛缩组织取自距舟关节内侧。使用Zip - tip C18消化和脱脂后提取蛋白质。使用化学发光分析法检测各个胶原组分。组织样本的氨基酸分析显示胶原蛋白占优势，即I型、III型和VI型胶原蛋白。甘氨酸和羟脯氨酸含量高表明I型和III型胶原蛋白占优势。共鉴定出19种细胞外基质蛋白。本研究的主要结果是观察到马蹄内翻足的细胞外基质由另外16种蛋白质组成，包括V型、VI型和XII型胶原蛋白，以及先前描述的I型和III型胶原蛋白和转化生长因子β。对马蹄内翻足不同区域细胞外基质的一般蛋白质组成进行表征可能有助于理解这种异常的发病机制，从而有助于开发更有效的治疗方法。
