Volkov E M, Poletaev G I
Biull Eksp Biol Med. 1983 Aug;96(8):33-5.
Experiments were carried out on phenotypically normal and sick homozygotic 129/Rej mice suffering from congenital dystrophy. The membrane rest potential (MRP) for muscle fibers of sick homozygotic animal diaphragm appeared lower than that of phenotypically normal species, attesting to the denervation-like pattern of muscle pathology. After muscle treatment with armine, and irreversible acetylcholinesterase inhibitor, d-tubocurarine chloride hyperpolarized the postsynaptic membrane without affecting the MRP of the extrasynaptic zone of muscle fibers. The magnitude of the postsynaptic hyperpolarization of the muscle membrane in response to curare turned out to be the same in both phenotypically normal and sick homozygotic mice. It is suggested that the genetic defect in question does not change the pattern of non-quantum acetylcholine secretion from the motor nerve endings. Therefore, this cannot be the reason for muscle pathology.
实验在患有先天性营养不良的表型正常和患病的纯合129/Rej小鼠身上进行。患病纯合动物膈肌肌肉纤维的膜静息电位（MRP）似乎低于表型正常的物种，这证明了肌肉病理的去神经样模式。在用不可逆的乙酰胆碱酯酶抑制剂阿米宁处理肌肉后，氯化d -筒箭毒碱使突触后膜超极化，而不影响肌肉纤维突触外区域的MRP。在表型正常和患病的纯合小鼠中，肌肉膜对箭毒的突触后超极化幅度是相同的。有人认为，所讨论的基因缺陷不会改变运动神经末梢非量子乙酰胆碱分泌的模式。因此，这不可能是肌肉病理的原因。
