Mc Laughlin D, Hajduk P, Murphy P, Puri P
National Children's Research Centre, Our Lady's Children's Hospital, Dublin, Ireland ; Children's University Hospital, Dublin, Ireland ; School of Natural Sciences, Trinity College Dublin, Dublin, Ireland.
Mol Syndromol. 2013 Feb;4(1-2):46-62. doi: 10.1159/000345579.
Animal models are of great importance for medical research. They have enabled analysis of the aetiology and pathogenesis of complex congenital malformations and have also led to major advances in the surgical and therapeutic management of these conditions. Animal models allow us to comprehend the morphological and molecular basis of disease and consequently to discover novel approaches for both surgical and medical therapy. The anthracycline antibiotic adriamycin was incidentally found to have teratogenic effects on rats, producing a range of defects remarkably similar to the VACTERL association of congenital anomalies in humans, providing a reproducible animal model of this condition. VACTERL association is a spectrum of birth defects which includes vertebral, anal, cardiovascular, tracheo-oesophageal, renal and limb anomalies. In recent years, adriamycin rodent models of VACTERL have provided valuable insights into the pathogenesis of this complex association, particularly in relation to tracheo-oesophageal malformations. The adriamycin rat model and adriamycin mouse model are now well established in the investigation of the morphology of faulty organogenesis and the regulation of gene expression in tracheo-oesophageal anomalies.
动物模型对医学研究至关重要。它们有助于分析复杂先天性畸形的病因和发病机制，也推动了这些疾病外科和治疗管理方面的重大进展。动物模型使我们能够理解疾病的形态学和分子基础，从而发现外科和医学治疗的新方法。蒽环类抗生素阿霉素偶然被发现对大鼠有致畸作用，产生一系列与人类先天性异常的VACTERL综合征非常相似的缺陷，提供了这种病症的可重复动物模型。VACTERL综合征是一系列出生缺陷，包括脊柱、肛门、心血管、气管食管、肾脏和肢体异常。近年来，VACTERL的阿霉素啮齿动物模型为这种复杂综合征的发病机制提供了有价值的见解，特别是在气管食管畸形方面。阿霉素大鼠模型和阿霉素小鼠模型现已在气管食管异常中器官发生缺陷的形态学和基因表达调控研究中得到充分确立。
