Wang Jianzhang, Du Juan, Song Yajuan, Tan Xiaoying, Wu Junzheng, Wang Tong, Shi Yi, Xu Xingbo, Yu Zhou, Song Baoqiang
Department of Plastic Surgery, Xijing Hospital, Fourth Military Medical University (Air Force Medical University), Xi'an, 710032, China.
Department of Dermatology, Xuanwu Hospital, Capital Medical University, Beijing, 100053, China.
Cell Death Dis. 2025 May 9;16(1):371. doi: 10.1038/s41419-025-07554-8.
Hypertrophic scar (HS) represents the most prevalent form of skin fibrosis, significantly impacting the quality of life. Despite this, the molecular mechanisms driving HS formation remain largely undefined, impeding the development of effective treatments. The study showed that Cartilage Intermediate Layer Protein 1 (CILP1) was predominantly expressed in myofibroblasts and was up-regulated in various forms of skin fibrosis, including human hypertrophic and keloid scars, and in animal models of HS. Notably, we detected elevated serum levels of CILP1 in fifty-two patients with HS compared to twenty healthy individuals, suggesting its potential as a novel biomarker. The findings indicated that CILP1 was involved in a negative feedback loop with TGF-β and inhibited the transcription of Peroxisome Proliferator-Activated Receptors (PPARs) via interaction with Y-box-binding protein 1 (YBX1). This interaction promoted cell proliferation, migration, and collagen production in hypertrophic scar fibroblasts (HSFs). In vivo studies further confirmed that CILP1 knockdown markedly reduced HS formation, whereas administration of recombinant human CILP1 protein exacerbated it. These discoveries illuminated the CILP1-YBX1-PPARs signaling pathway as a key regulator of HS formation, offering a foundation for novel therapeutic approaches.
增生性瘢痕(HS)是皮肤纤维化最常见的形式,严重影响生活质量。尽管如此,驱动HS形成的分子机制在很大程度上仍不明确,这阻碍了有效治疗方法的开发。研究表明,软骨中间层蛋白1(CILP1)主要在肌成纤维细胞中表达,并且在各种形式的皮肤纤维化中上调,包括人类增生性瘢痕和瘢痕疙瘩,以及HS动物模型。值得注意的是,与20名健康个体相比,我们在52名HS患者中检测到血清CILP1水平升高,表明其作为一种新型生物标志物的潜力。研究结果表明,CILP1与转化生长因子-β(TGF-β)形成负反馈回路,并通过与Y盒结合蛋白1(YBX1)相互作用抑制过氧化物酶体增殖物激活受体(PPARs)的转录。这种相互作用促进了增生性瘢痕成纤维细胞(HSFs)的细胞增殖、迁移和胶原蛋白生成。体内研究进一步证实,敲低CILP1可显著减少HS的形成,而给予重组人CILP1蛋白则会使其加重。这些发现阐明了CILP1-YBX1-PPARs信号通路是HS形成的关键调节因子,为新的治疗方法提供了基础。