Animal models have contributed greatly to our understanding of human diseases. Here, we focus on cornea epithelial stem cell (CESC) deficiency (commonly called limbal stem cell deficiency, LSCD). Corneal development, homeostasis and wound healing are supported by specific stem cells, that include the CESCs. Damage to or loss of these cells results in blindness and other debilitating ocular conditions. Here we describe the contributions from several vertebrate models toward understanding CESCs and LSCD treatments. These include both mammalian models, as well as two aquatic models, Zebrafish and the amphibian, Xenopus. Pioneering developments have been made using stem cell transplants to restore normal vision in patients with LSCD, but questions still remain about the basic biology of CESCs, including their precise cell lineages and behavior in the cornea. We describe various cell lineage tracing studies to follow their patterns of division, and the fates of their progeny during development, homeostasis, and wound healing. In addition, we present some preliminary results using the Xenopus model system. Ultimately, a more thorough understanding of these cornea cells will advance our knowledge of stem cell biology and lead to better cornea disease therapeutics.

中文翻译：

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了解角膜上皮干细胞和干细胞缺乏症：使用脊椎动物模型系统的经验教训

动物模型极大地促进了我们对人类疾病的理解。在这里，我们关注角膜上皮干细胞 (CESC) 缺乏症（通常称为角膜缘干细胞缺乏症，LSCD）。包括 CESC 在内的特定干细胞支持角膜发育、稳态和伤口愈合。这些细胞的损伤或丢失会导致失明和其他使人衰弱的眼部疾病。在这里，我们描述了几种脊椎动物模型对理解 CESC 和 LSCD 治疗的贡献。其中包括哺乳动物模型以及两种水生模型斑马鱼和两栖动物非洲爪蟾. 使用干细胞移植来恢复 LSCD 患者的正常视力已经取得了开创性的进展，但关于 CESC 的基本生物学问题仍然存在，包括它们在角膜中的精确细胞谱系和行为。我们描述了各种细胞谱系追踪研究，以跟踪它们的分裂模式，以及它们在发育、体内平衡和伤口愈合过程中后代的命运。此外，我们展示了一些使用非洲爪蟾模型系统的初步结果。最终，对这些角膜细胞的更透彻的了解将推进我们对干细胞生物学的了解，并导致更好的角膜疾病治疗。