The Ataxic Syrian Hamster: An Animal Model Homologous to the pcd Mutant Mouse?
The Ataxic Syrian Hamster: An Animal Model Homologous to the pcd Mutant Mouse?
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DOI：
10.1007/s12311-009-0113-9
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发表时间：
2009-09-01
期刊：
影响因子：
3.5
通讯作者：
Arai, Shigeyuki
中科院分区：
文献类型：
--
作者：
Akita, Kenji;Arai, Shigeyuki
本文描述了一种自发的叙利亚仓鼠小脑共济失调模型。育种数据表明，条件是遗传和遗传方式是常染色体隐性。纯合子比野生型小，但外观正常。突变体在7周龄时开始表现出中度共济失调。虽然受影响的成年人表现出明显的小脑萎缩，但通过光学显微镜观察，大脑的其他部分似乎相对正常。突变体在18月龄时几乎失去所有的浦肯野细胞，颗粒细胞密度中度降低，可能是浦肯野细胞原发性丢失的结果。在纯合子仓鼠脑中，Nna 1表达被抑制，类似于先前在浦肯野细胞变性（pcd）突变小鼠中观察到的。共济失调仓鼠与pcd突变小鼠的表型比较表明，共济失调仓鼠中致病等位基因的影响比突变小鼠中发现的大多数等位基因要温和得多。
A spontaneous model of cerebellar ataxia in the Syrian hamster is described. Breeding data indicate that the condition is hereditary and that the mode of inheritance is autosomal recessive. Homozygotes are smaller in size than the wild-type but have a normal appearance. Mutants show a moderate ataxia beginning at 7 weeks of age. Although affected adults exhibit significant atrophy in the cerebellum, other parts of the brain appear relatively normal by light microscopy. Mutants lose almost all Purkinje cells by 18 months of age and exhibit a moderate reduction in granule cell density, probably as a consequence of the primary loss of Purkinje cells. In the homozygous hamster brain, Nna1 expression is suppressed, similar to that previously observed in Purkinje cell degeneration (pcd) mutant mice. A phenotypic comparison of ataxic hamsters with the pcd mutant mice suggests that the influence of the causal allele in ataxic hamsters is considerably milder than most of the alleles found in the mutant mice.