Herbik Magdalena A, Chrapusta Stanisław J, Kowalczyk Anna, Grieb Paweł
Department of Experimental Pharmacology, Polish Academy of Sciences Medical Research Centre, 5 Pawińskiego St, 02-106 Warsaw, Poland.
Folia Neuropathol. 2006;44(3):149-53.
A colony of transgenic rats expressing the human mutant Cu,Zn superoxide dismutase gene (hSOD1G93A) that is associated with some cases of familial form of amyotrophic lateral sclerosis (ALS) has been maintained in the Animal House of the Polish Academy of Sciences Medical Research Centre since 2003. This transgenic model, generated by Howland et al. (Proc Natl Acad Sci USA 2002; 99: 1604-1609), has been obtained under the material transfer agreement from Wyeth Corporation. The transgenic SOD1G93A (or 'Howland') rats develop neurological and neuropathological symptoms reminiscent of human ALS, i.e. progressive loss of motoneurons leading to paralysis and death. This paper describes maintenance of the transgenic rat colony, and general procedures used in experiments with these animals (i.e. genotyping, neurological observations, anaesthesia, etc.). At the beginning of the colony, up to the 3rd generation of the rats, symptoms of the model disease appeared at 95-125 days of age, and the animals survived till 120-145 days of age. Thereafter a gradual change in the disease phenotype occurred, and in the 8th generation approximately 1/3 of the rats displayed much slowed disease progression.
自2003年起，一群表达与某些家族性肌萎缩侧索硬化症（ALS）病例相关的人类突变型铜锌超氧化物歧化酶基因（hSOD1G93A）的转基因大鼠被饲养在波兰科学院医学研究中心的动物房。这个由豪兰等人（《美国国家科学院院刊》2002年；99：1604 - 1609）构建的转基因模型，是根据与惠氏公司的材料转让协议获得的。转基因SOD1G93A（或“豪兰”）大鼠会出现类似于人类ALS的神经学和神经病理学症状，即运动神经元逐渐丧失导致瘫痪和死亡。本文描述了转基因大鼠群体的饲养情况，以及在这些动物实验中使用的一般程序（即基因分型、神经学观察、麻醉等）。在群体开始时，直到第3代大鼠，模型疾病的症状在95 - 125日龄出现，动物存活到120 - 145日龄。此后疾病表型逐渐发生变化，在第8代时，约1/3的大鼠疾病进展明显减缓。
