SITE OF PCD GENE ACTION AND PURKINJE-CELL MOSAICISM IN CEREBELLA OF CHIMAERIC MICE
SITE OF PCD GENE ACTION AND PURKINJE-CELL MOSAICISM IN CEREBELLA OF CHIMAERIC MICE
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DOI：
10.1038/270245a0
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发表时间：
1977-01-01
期刊：
影响因子：
64.8
通讯作者：
MULLEN, RJ
中科院分区：
文献类型：
--
作者：
MULLEN, RJ
PURKINJE细胞变性（pcd）是一种新的小鼠常染色体隐性遗传突变。受影响的动物的特点是中度共济失调，这是由于出生后几乎所有小脑浦肯野细胞的损失1。我们在这里描述的实验，以确定是否pcd基因的行为内在的浦肯野细胞或是否退变是继发于原发性病变的一些其他类型的细胞。在这项研究中，实验嵌合体含有突变（pcd/pcd）和基因型正常（+/+）细胞的混合物（这些嵌合体表示为pcd/pcd←→+/+）。结果表明，pcd基因在浦肯野细胞内起作用。嵌合体也被用于检查+/+浦肯野细胞的存活群体，以获得克隆发育的证据。令人惊讶的是，这些初步研究没有发现克隆的证据;来自+/+组分的细胞的分布似乎是随机的。
PURKINJE cell degeneration,pcd, is a new autosomal recessive mutation in mice. Affected animals are characterised by a moderate ataxia that results from the postnatal loss of virtually all cerebellar Purkinje cells1. We describe here experiments to determine whether thepcdgene acts intrinsically within the Purkinje cell or whether the degeneration is secondary to a primary lesion in some other cell type. In this study, experimental chimaeras were made which contained mixtures of mutant (pcd/pcd) and genotypically normal (+/+) cells (these chimaeras are denotedpcd/pcd←→+/+). The results indicate that thepcdgene does act within the Purkinje cell. The chimaeras have also been used to examine the surviving population of +/+ Purkinje cells for evidence of clonal development. Surprisingly, these preliminary studies reveal no evidence of clones; the distribution of cells derived from the +/+ component seems random.