Lev A A, Feener C C, Kunkel L M, Brown R H
Day Neuromuscular Research Center, Massachusetts General Hospital, Boston 02114.
J Biol Chem. 1987 Nov 25;262(33):15817-20.
A partial cDNA clone for the Duchenne's muscular dystrophy (DMD) locus was used to investigate the expression of this locus in human muscle in vitro. Hybridization to a 14-kilobase RNA transcript was demonstrated in both fetal and mature human skeletal muscle and four lines of human muscle cells in culture. The DMD transcript was not detected in cultured cells outside the muscle lineage. In cultured muscle cells, gene expression was evident only in myotubes both before and after innervation with mouse spinal cord. Primary cultures of human myoblasts did not show the presence of the DMD transcript prior to fusion to form myotubes. An in vitro model is potentially an excellent system in which to investigate factors controlling expression of the DMD gene in normal muscle and how this expression is altered in cultured DMD muscle.
利用杜兴氏肌营养不良(DMD)基因座的部分cDNA克隆,在体外研究该基因座在人肌肉中的表达。在胎儿和成熟的人骨骼肌以及四种培养的人肌肉细胞系中,均证实了与14千碱基RNA转录本的杂交。在肌肉谱系以外的培养细胞中未检测到DMD转录本。在培养的肌肉细胞中,仅在小鼠脊髓支配前后的肌管中基因表达明显。人成肌细胞的原代培养物在融合形成肌管之前未显示DMD转录本的存在。体外模型可能是一个极好的系统,可用于研究控制正常肌肉中DMD基因表达的因素,以及在培养的DMD肌肉中这种表达是如何改变的。