Establishment of a novel dwarf rat strain: cartilage calcification insufficient (CCI) rats.
Establishment of a novel dwarf rat strain: cartilage calcification insufficient (CCI) rats.
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建立一种新型矮人大鼠菌株：软骨钙化不足（CCI）大鼠。
DOI：
10.1538/expanim.14-0072
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发表时间：
2015
影响因子：
2.4
通讯作者：
Tanuma J
中科院分区：
文献类型：
--
作者：
Tanaka M;Watanabe M;Yokomi I;Matsumoto N;Sudo K;Satoh H;Igarashi T;Seki A;Amano H;Ohura K;Ryu K;Shibata S;Nagayama M;Tanuma J
在St. Marianna University Graduate School of Medicine动物实验研究所饲养的Jcl衍生Sprague-Dawley大鼠群中，出现了伴有骨骼异常（如四肢、尾巴和身体短小）的侏儒症大鼠（侏儒大鼠）。由于侏儒症被认为是由于基于其频率的基因突变，我们饲养侏儒大鼠，并研究它们的特征，以确定其表型的致病因素，以及它们是否可以用作人类疾病模型。选择一只雄性和一只雌性产生侏儒后代，通过配对开始繁殖。侏儒症的发病率为25.8%，其中产生的窝，侏儒症发生在两个性别，这表明它是一个常染色体隐性遗传方式。12周龄时，雄性和雌性侏儒大鼠的体重分别为正常大鼠的40%和57%。在软X射线影像学和组织学检查中，观察到长骨（如胫骨和股骨）缩短和发育不全，提示软骨内骨化异常。免疫组织化学检查发现聚集蛋白聚糖合成障碍，这可能导致延迟钙化和生长板增厚的侏儒大鼠。我们假设侏儒大鼠的主要特征是由其长骨中的软骨钙化不足引起的，因此，我们将其命名为软骨钙化不足（CCI）大鼠。
Rats with dwarfism accompanied by skeletal abnormalities, such as shortness of the limbs, tail, and body (dwarf rats), emerged in a Jcl-derived Sprague-Dawley rat colony maintained at the Institute for Animal Experimentation, St. Marianna University Graduate School of Medicine. Since the dwarfism was assumed to be due to a genetic mutation based on its frequency, we bred the dwarf rats and investigated their characteristics in order to identify the causative factors of their phenotypes and whether they could be used as a human disease model. One male and female that produced dwarf progeny were selected, and reproduction was initiated by mating the pair. The incidence of dwarfism was 25.8% among the resultant litter, and dwarfism occurred in both genders, suggesting that it was inherited in an autosomal recessive manner. At 12 weeks of age, the body weights of the male and female dwarf rats were 40% and 57% of those of the normal rats, respectively. In soft X-ray radiographic and histological examinations, shortening and hypoplasia of the long bones, such as the tibia and femur, were observed, which were suggestive of endochondral ossification abnormalities. An immunohistochemical examination detected an aggrecan synthesis disorder, which might have led to delayed calcification and increased growth plate thickening in the dwarf rats. We hypothesized that the principal characteristics of the dwarf rats were systemically induced by insufficient cartilage calcification in their long bones; thus, we named them cartilage calcification insufficient (CCI) rats.