Sakamoto A, Abe M, Masaki T
Department of Bioscience, Research Institute, National Cardiovascular Center, Suita, Osaka, Japan.
FEBS Lett. 1999 Mar 19;447(1):124-8. doi: 10.1016/s0014-5793(99)00267-7.
Cardiomyopathic hamster is a representative animal model for autosomal recessive cardiomyopathy. We have previously shown that the transcript of delta-sarcoglycan is missing in the heart of cardiomyopathic hamster due to genomic deletion. Here we define the normal genomic region deleted in cardiomyopathic hamster, which spans about 30 kb interval and includes the two first exons of the delta-sarcoglycan gene. RNA blot analysis using genomic DNA fragments covering the entire deletion as probes failed to detect any transcript other than delta-sarcoglycan in normal hamster heart, suggesting that delta-sarcoglycan is the only transcript defective in the heart of cardiomyopathic hamster.
心肌病仓鼠是常染色体隐性心肌病的代表性动物模型。我们之前已经表明，由于基因组缺失，心肌病仓鼠心脏中δ-肌聚糖的转录本缺失。在此，我们确定了心肌病仓鼠中缺失的正常基因组区域，该区域跨度约30 kb，包括δ-肌聚糖基因的前两个外显子。使用覆盖整个缺失区域的基因组DNA片段作为探针进行RNA印迹分析，未能在正常仓鼠心脏中检测到除δ-肌聚糖以外的任何转录本，这表明δ-肌聚糖是心肌病仓鼠心脏中唯一有缺陷的转录本。
