DEVELOPMENTAL CONTROL OF THE EXCITABILITY OF MUSCLE - TRANSPLANTATION EXPERIMENTS ON A MYOTONIC MOUSE MUTANT
DEVELOPMENTAL CONTROL OF THE EXCITABILITY OF MUSCLE - TRANSPLANTATION EXPERIMENTS ON A MYOTONIC MOUSE MUTANT
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DOI：
10.1073/pnas.85.11.3880
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发表时间：
1988-06-01
影响因子：
11.1
通讯作者：
JOCKUSCH, H
中科院分区：
文献类型：
--
作者：
FUCHTBAUER, EM;REININGHAUS, J;JOCKUSCH, H
研究了肌强直动物模型的发育方面，即称为“翻正反应发育停滞”（ADR 表型）的小鼠突变体。成人 ADR 肌肉的特点是膜的氯电导率低，导致过度兴奋，并且小白蛋白含量低。 ADR 肌肉的强直性过度兴奋性（通过“后收缩”程度来衡量）在出生后第 9 天到第 18 天之间急剧增加，此时已接近成人水平。为了研究强直表型的组织自主性，在 ADR 和野生型（WT 表型）供体和宿主之间的所有四种组合中进行肌肉移植。在大多数实验中，供体和宿主对再生肌肉的相对贡献是由等位基因标记（磷酸葡萄糖异构酶）决定的。在WT和ADR宿主中，ADR移植物表现出肌强直反应，而在WT裸鼠宿主中，ADR移植物表现出不完整的肌强直反应，并且与幼年ADR肌肉的肌强直反应相似。 WT 供体的移植物在任何情况下都没有表现出任何肌强直。这表明强直性 ADR 表型是基于最有可能与质膜相关的内在肌肉特性。与未移植肌肉的小清蛋白含量相比，移植肌肉的小白蛋白含量表明这种次要表型特性的表达中存在移植物-宿主相互作用。
Developmental aspects of an animal model of myotonia, the mouse mutant called "arrested development of righting response" (ADR phenotype), were studied. Adult ADR muscle is characterized by a low chloride conductance of the membrane, leading to hyperexcitability, and by a low parvalbumin content. The myotonic hyperexcitability (as measured by the extent of "aftercontractions") of ADR muscle increased steeply between postnatal days 9 and 18, by which time it had approached the adult level. To study the tissue autonomy of the myotonic phenotype, muscle grafts were performed in all four combinations between ADR and wildtype (WT phenotype) donors and hosts. In most experiments, the relative contributions of donor and host to the regenerated muscles were determined by an allelic marker (glucose phosphate isomerase). In WT and ADR hosts, ADR grafts showed myotonic responses that in WT nude mouse hosts were incomplete and similar to those of juvenile ADR muscle. In no case did grafts from WT donors show any myotonia. This shows that the myotonic ADR phenotype is based on an intrinsic muscle property most likely related to the plasma membrane. The parvalbumin contents of grafted muscles, when compared with those of untransplanted muscles, indicated graft-host interaction in the expression of this secondary phenotypic property.