Rabkin S W, Cheng K M
Cardiovascular Research Laboratory, University Hospital (Shaughnessy), Vancouver, Canada.
Basic Res Cardiol. 1992 Nov-Dec;87(6):610-7. doi: 10.1007/BF00788671.
A new genetic cardiomyopathy was identified in a blind mutant avian strain. Cardiac myocytes were cultured from 7-day-old chick embryos from Rhode Island Red chickens and from another strain of this species that has been identified to have several abnormalities, the most striking of which is blindness. Cardiac myocytes were maintained in tissue culture. Morphologically, in culture, the cardiac myocyte from the blind mutant strain assumed a spherical shape and showed abnormalities of sarcolemmal membrane compared to control myocytes from heterozygous animals. Choline uptake and metabolism were examined, using [methyl 3H] choline, because it is a sarcolemmal transport process and choline is metabolised to phosphatidylcholine, an important phospholipid for cellular structure and function. Choline uptake through the active transport process was markedly and significantly reduced in the mutant cells compared to control cells, while choline metabolism to phosphatidylcholine was not significantly altered. These results demonstrate a new abnormality of cardiac myocytes, a cardiomyopathy that can be studied in cell culture and one with abnormalities of cellular choline transport.
在一个盲突变禽类品系中发现了一种新的遗传性心肌病。从罗德岛红鸡的7日龄鸡胚以及该物种的另一个已被鉴定有多种异常（其中最显著的是失明）的品系中培养心肌细胞。心肌细胞在组织培养中维持生长。在形态学上，与来自杂合动物的对照心肌细胞相比，来自盲突变品系的培养心肌细胞呈球形，并且肌膜出现异常。使用[甲基³H]胆碱检测胆碱摄取和代谢，因为这是一种肌膜转运过程，且胆碱会代谢为磷脂酰胆碱，后者是一种对细胞结构和功能很重要的磷脂。与对照细胞相比，突变细胞中通过主动转运过程的胆碱摄取显著且明显减少，而胆碱代谢为磷脂酰胆碱的过程没有显著改变。这些结果表明心肌细胞出现了一种新的异常，即一种可在细胞培养中研究的心肌病，且存在细胞胆碱转运异常。
