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首页> 外文期刊>Proceedings of the National Academy of Sciences of the United States of America >Attractin/Mahogany/Zitter plays a critical role in myelination of the central nervous system
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Attractin/Mahogany/Zitter plays a critical role in myelination of the central nervous system

机译：Attractin /桃花心木/ Zitter在中枢神经系统的髓鞘形成中起关键作用

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摘要

The rat zitter (zi) mutation induces hypomyelination and vacuola- tion in the central nervous system (CNS), which result in early-onset tremor and progressive flaccid paresis. By positional cloning, we found a marked decrease in Attractin (Atrn) mRNA in the brain of the zi/zi rat and identified zi as an 8-bp deIetion at a spIice donor site of Atrn. Atrn has been known to play multiple roles in regulating physiological processes that are involved in monocyte--T cell interaction, agouti-related hair pigmentation, and control of energy homeostasis. Rat Atrn gene encoded two isoforms, a secreted and a membrane form, as a result of alternative splicing. The zi mutation at the Atffi locus darkened coat color when introduced into agouti rats, as also described in mahogany (mg) mice, carrying the homozygous mutation at the Atrn locus. Trans- genic rescue experiments showed that the membrane-type Atrn complemented both neurological alteration and abnormal pigmen- tation in zi/zi rats, but that the secreted-type Atrn complemented neither mutant phenotype. Furthermore, we discovered that mg mice exhibited hypomyelination and vacuolation in the CNS asso- cited with body tremor. We conclude from these results that the membrane Atrn has a critical role in normal myelination in the CNS and would provide insights into the physiology of myelination as well as the etiology of myelin diseases.
机译：大鼠zitter（zi）突变会引起中枢神经系统（CNS）的髓鞘过少和空泡化，从而导致早发性震颤和进行性松弛性麻痹。通过位置克隆，我们发现zi / zi大鼠大脑中的Attractin（Atrn）mRNA显着降低，并将zi鉴定为Atrn的一个供体位点的8 bp决定。已知Atrn在调节涉及单核细胞-T细胞相互作用，与刺古龙相关的头发色素沉着和控制能量稳态的生理过程中扮演多个角色。由于选择性剪接，Rat Atrn基因编码两种同种型，一种是分泌型，一种是膜型。如引入桃花心木（mg）小鼠中所述，将Atffi基因座处的zi突变加到豚鼠中时，其外套颜色变暗，在Atrn基因座处携带纯合突变。转基因抢救实验表明，膜型Atrn补充了zi / zi大鼠的神经系统改变和异常色素，但分泌型Atrn既不补充突变表型。此外，我们发现mg小鼠在与身体震颤有关的CNS中表现出髓鞘过少和空泡化。我们从这些结果得出结论，膜Atrn在中枢神经系统的正常髓鞘形成中起着关键作用，并且将提供对髓鞘形成的生理学以及髓鞘疾病的病因学的见识。

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