ELECTRICAL MYOTONIA AND CATARACT IN X-LINKED MUSCULAR-DYSTROPHY (MDX) MOUSE
ELECTRICAL MYOTONIA AND CATARACT IN X-LINKED MUSCULAR-DYSTROPHY (MDX) MOUSE
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DOI：
10.1016/0022-510x(90)90202-x
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发表时间：
1990-10-01
影响因子：
4.4
通讯作者：
KINOSHITA, M
中科院分区：
文献类型：
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作者：
KURIHARA, T;KISHI, M;KINOSHITA, M
X染色体连锁突变小鼠（mdx）已被研究作为杜氏肌营养不良症的动物模型，并已发现在肌肉表面膜中具有相同的肌营养不良蛋白缺陷。mdx小鼠半侧膈肌制备物的细胞内记录显示在微电极插入和撤回时发生的低静息膜电位和电性肌强直。在低温下，在30-50%的刺穿的肌纤维中观察到mdx小鼠的电性肌强直，在37 ℃下，其降低到仅7.8%。C.（+）-筒箭毒碱不能消除mdx的电性肌强直。虽然mdx小鼠没有行为性肌强直，但mdx小鼠动作电位的重复爆发是基于肌膜的异常，因为神经肌肉阻滞不能消除重复爆发。此外，对mdx小鼠晶状体的密切观察揭示了从新生期到成年期的白内障。对mdx小鼠晶状体的裂隙灯检查显示，随着它们的生长，核性白内障随后是前囊下白内障。mdx小鼠的白内障与强直性肌营养不良的白内障不同，强直性肌营养不良的白内障通常位于后囊下。
An X chromosome-linked mouse mutant (mdx) has been investigated as an animal model of Duchenne''s muscular dystrophy, and has been found to have the same defect of dystrophin in the muscle surface membrane. Intracellular recordings from the mdx mouse hemidiaphragm preparations revealed low resting membrane potentials and electrical myotonia which occurred at the time of microelectrode insertion and withdrawal. Electrical myotonia of the mdx mouse was observed in 30-50% of the impaled muscle fibers at low temperature, which decreased to only 7.8% at 37.degree. C. Electrical myotonia of mdx was not abolished by (+)-tubocurarine. Though there was no behavioral myotonia in mdx mice, repetitive bursts of action potentials in mdx mice were based on the abnormalities of the muscle membrane since neuromuscular blockade did not abolish the repetitive bursts. Also, close observation of the lenses of mdx mice revealed cataracts from the newborn stage to the adult age. Slit lamp examination of the lenses of the mdx mice revealed nuclear cataracts followed by anterior subcapsular cataract as they grew. The cataract of mdx mice is different from that of myotonic dystrophy which is usually posterior subcapsular.