ISOFORM-SELECTIVE DEFICIT OF GLYCINE RECEPTORS IN THE MOUSE MUTANT SPASTIC
ISOFORM-SELECTIVE DEFICIT OF GLYCINE RECEPTORS IN THE MOUSE MUTANT SPASTIC
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DOI：
10.1016/0896-6273(92)90295-o
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发表时间：
1992-02-01
期刊：
影响因子：
16.2
通讯作者：
BETZ, H
中科院分区：
文献类型：
--
作者：
BECKER, CM;SCHMIEDEN, V;BETZ, H
突变小鼠痉挛（spa）在出生后约2周出现特征性运动障碍，症状类似于甘氨酸能拮抗剂士的宁亚致死中毒。相应地，成年纯合子突变体（spa/spa）在脊髓和大脑中表现出严重的抑制性甘氨酸受体减少。在这里，我们表明痉挛突变选择性地干扰出生后甘氨酸受体蛋白成人异构体的积累，而围产期新生儿受体异构体的表达不受明显影响。poly(A)+ RNA的异源表达和Northern blot分析表明，成年痉挛突变体脊髓中甘氨酸受体α -1亚基转录物水平正常。因此，出生后痉挛症状的年龄依赖性表现反映了突变对成人甘氨酸受体异构体发育表达的选择性影响。
The mutant mouse spastic (spa) develops a characteristic motor disorder about 2 weeks after birth, with symptoms resembling sublethal poisoning by the glycinergic antagonist strychnine. Correspondingly, adult homozygotic mutants (spa/spa) exhibit a severe reduction of inhibitory glycine receptors in spinal cord and brain. Here we show that the spastic mutation selectively interferes with the postnatal accumulation of the adult isoform of the glycine receptor protein, whereas perinatal expression of the neonatal receptor isoform is not detectably affected. Heterologous expression in X. laevis oocytes of poly(A)+ RNA and Northern blot analysis indicate normal levels of glycine receptor alpha-1 subunit transcripts in spinal cord of adult spastic mutants. Thus, the age-dependent manifestation of spastic symptoms after birth reflects a selective effect of the mutation on the developmental expression of the adult glycine receptor isoform.