A murine model of cystic fibrosis.
A murine model of cystic fibrosis.
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囊性纤维化的小鼠模型。
DOI：
10.1164/ajrccm/151.3_pt_2.s59
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发表时间：
1995
期刊：
影响因子：
--
通讯作者：
Koller,BH
中科院分区：
文献类型：
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作者：
Snouwaert,JN;Brigman,KK;Latour,AM;Iraj,E;Schwab,U;Gilmour,MI;Koller,BH
我们之前报道过，对CFTR （i -）小鼠后代的遗传分析表明，没有与S489X突变相关的产前死亡率，因为缺乏S489X突变的后代与突变的杂合或纯合后代的比例并没有明显偏离预期的孟德尔比例1:2:1。然而，我们发现80%至90%的CFTR （i -）小鼠在出生后发育的两个时期死亡。第一个时期包括出生后发育的前5天。在这一时期存活下来的大多数动物在20天内仍然存活。然而，大部分存活的CFTR（-1）小鼠在出生后20至27天之间死亡，这段时间是小鼠正常断奶的时间。在我们最初报告时，只有一只CFTR（-1）动物存活了40天。除了平均寿命大幅缩短外，我们的许多CFTR （i - 1）小鼠的产后生长也有所下降，有些动物的体重比对照组的幼崽轻50%。在我们随后的分析中，我们最初报道的CFTR（-1-）小鼠的生长和存活模式仍然成立。然而，我们发现，一旦过了关键的断奶期，我们的CFTR（-1-）小鼠往往能存活很长时间，然后因肠梗阻而死亡。因此，通过增加我们繁殖群体的规模，我们已经能够获得相当数量的这些年长的cftr （i -）动物，其中一些已经存活了一年多。
RESULTS Survival We reported previously that genetic analysis of the offspring of CFTR (-I-) mice indicated that there was no prenatal mortality associated with the S489X mutation, because the ratio of offspring lacking the S489X mutation to those heterozygous or homozygous for the mutation did not deviate significantly from the expected Mendelian ratio of 1: 2: 1. However, we found that 80 to 90% of the CFTR (-I-) mice died during two periods of postnatal development. The first such period encompassed the first 5 days of postnatal development. The majority of the animals that survived this period were still alive at 20 days. However, a large proportion of the surviving CFTR (-1-) animals died between postnatal days 20 and 27, the period during which mice are normally weaned. At the time of our initial report, only one CFTR (-1-) animal had survived past 40 days. In addition to a drastically shortened average life span, many of our CFTR (-I-) mice showed decreased postnatal growth, with some animals weighing 50% less than their control littermates.In our subsequent analyses, the patterns of growth and survival that we originally reported for our CFTR (-1-) mice have continued to hold true. However, we have found that, once they have passed through the critical weaning period, our CFTR (-1-) mice often remain alive for a long time before succumbing to death by intestinal obstruction. Thus, by increasing the size of our breeding colony, we have been able to obtain a substantial number of these olderCFTR (-I-) animals, some of which have survived for more than a year.