Mossbrugger I, Hoelzlwimmer G, Calzada-Wack J, Quintanilla-Martinez L
Institut für Pathologie-GSF-Forschungszentrum für Umwelt und Gesundheit, Neuherberg.
Verh Dtsch Ges Pathol. 2007;91:98-103.
Inbred strains are the raw material for the generation of Genetically Engineered Mice (GEM) that have become indispensable tools for cancer research, and for the identification of genes involved in human diseases. The "German Mouse Clinic" was designed to provide the scientific community with a systematic, standardized and comprehensive phenotyping of mouse models on various genetic backgrounds and generated by different methods (transgenic, knockouts, ENU mutagenesis screen and gene-trap approaches). The pathology screen within the German Mouse-Clinic was conceived to ensure a complete morphologic phenotype of mouse models, to support discovery of genes functions, and to understand how these genes influence the development of human diseases. The goal is to define disease entities that can be recognized by a pathologist and relate them to human disorders when possible. Knowing the inherent morphologic phenotype of the most frequent used mouse strains is of utmost importance for the correct interpretation of mouse models. The main challenges, which pathologist are confronted to validate mouse models for human diseases include (1) knowledge of mouse biology and of histological differences between mouse strains and humans, (2) the terminology that should be used for the classification of neoplastic lesions in GEM's, (3) to asses the usefulness of a particular GEM as model for a human disease.
近交系是培育基因工程小鼠（GEM）的原材料，基因工程小鼠已成为癌症研究以及鉴定人类疾病相关基因不可或缺的工具。“德国小鼠诊所”旨在为科学界提供对各种遗传背景且通过不同方法（转基因、基因敲除、ENU诱变筛选和基因捕获方法）产生的小鼠模型进行系统、标准化和全面的表型分析。德国小鼠诊所内的病理学筛查旨在确保小鼠模型具有完整的形态学表型，支持发现基因功能，并了解这些基因如何影响人类疾病的发展。目标是定义病理学家能够识别的疾病实体，并在可能的情况下将它们与人类疾病联系起来。了解最常用小鼠品系的固有形态学表型对于正确解释小鼠模型至关重要。病理学家在验证人类疾病小鼠模型时面临的主要挑战包括：（1）了解小鼠生物学以及小鼠品系与人类之间的组织学差异；（2）用于对基因工程小鼠中的肿瘤性病变进行分类的术语；（3）评估特定基因工程小鼠作为人类疾病模型的实用性。
