Nair Rohini M, Kaliki Swathi, Vemuganti Geeta K
School of Medical Sciences, University of Hyderabad, Hyderabad, India.
Saudi J Ophthalmol. 2013 Jul;27(3):141-6. doi: 10.1016/j.sjopt.2013.06.008.
Advances in animal models of retinoblastoma have accelerated research in this field, aiding in understanding tumor progression and assessing therapeutic modalities. The distinct pattern of mutations and specific location of this unique intraocular tumor have paved the way for two types of models- those based on genetic mutations, and xenograft models. Retinoblastoma gene knockouts with an additional loss of p107, p130, p53 and using promoters of Nestin, Chx10, and Pax6 genes show histological phenotypic changes close to the human form of retinoblastoma. Conditional knockout in specific layers of the developing retina has thrown light on the origin of this tumor. The use of xenograft models has overcome the obstacle of time delay in the presentation of symptoms, which remains a crucial drawback of genetic models. With the advances in molecular and imaging technologies, the current research aims to develop models that mimic all the features of retinoblastoma inclusive of its initiation, progression and metastasis. The combination of genetic and xenograft models in retinoblastoma research has and will help to pave way for better understanding of retinoblastoma tumor biology and also in designing and testing effective diagnostic and treatment modalities.
视网膜母细胞瘤动物模型的进展加速了该领域的研究,有助于了解肿瘤进展并评估治疗方式。这种独特的眼内肿瘤的不同突变模式和特定位置为两种模型奠定了基础——基于基因突变的模型和异种移植模型。视网膜母细胞瘤基因敲除同时伴有p107、p130、p53缺失,并使用巢蛋白、Chx10和Pax6基因的启动子,显示出接近人类视网膜母细胞瘤形式的组织学表型变化。在发育中的视网膜特定层进行条件性敲除揭示了这种肿瘤的起源。异种移植模型的使用克服了症状出现时间延迟的障碍,这仍然是基因模型的一个关键缺点。随着分子和成像技术的进步,当前的研究旨在开发能够模拟视网膜母细胞瘤所有特征(包括其起始、进展和转移)的模型。视网膜母细胞瘤研究中基因模型和异种移植模型的结合已经并将有助于更好地理解视网膜母细胞瘤肿瘤生物学,也有助于设计和测试有效的诊断和治疗方式。