Survival of, and competition between, oligodendrocytes expressing different alleles of the Plp gene.
Survival of, and competition between, oligodendrocytes expressing different alleles of the Plp gene.
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DOI：
10.1083/jcb.200202124
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发表时间：
2002-08-19
期刊：
影响因子：
--
通讯作者：
Griffiths IR
中科院分区：
文献类型：
--
作者：
Edgar JM;Anderson TJ;Dickinson PJ;Barrie JA;McCulloch MC;Nave KA;Griffiths IR
x连锁Plp基因的突变导致髓鞘异常表型和少突胶质细胞死亡。在这里，我们利用X失活现象来证明不同突变Plp等位基因对少突胶质细胞存活的影响存在层次关系。我们使用复合杂合子小鼠来研究表达jimpy或rumpshaker等位基因的少突胶质细胞在表达Plp-null等位基因的背景下的长期命运。虽然突变少突胶质细胞和无突变少突胶质细胞数量相等，但表达突变等位基因的比例随后下降，而表达摇臀等位基因的群体数量减少但稳定，但畸形细胞的数量逐渐下降。中枢神经系统不同区域的跳跃细胞衰退的年龄与髓鞘形成的时间顺序相关。在表达摇臀和摇臀等位基因的复合杂合子中，表达摇臀和摇臀等位基因的少突胶质细胞占主导地位，并且比摇臀和无等位基因竞争时更丰富。因此，少突胶质细胞的存活并不仅仅取决于细胞的内在因素，如错误折叠的PLP的构象，而是受到邻近细胞的影响，可能会竞争细胞存活因子。
Mutations in the X-linked Plp gene lead to dysmyelinating phenotypes and oligodendrocyte cell death. Here, we exploit the X inactivation phenomenon to show that a hierarchy exists in the influence of different mutant Plp alleles on oligodendrocyte survival. We used compound heterozygote mice to study the long-term fate of oligodendrocytes expressing either the jimpy or rumpshaker allele against a background of cells expressing a Plp-null allele. Although mutant and null oligodendrocytes were generated in equal numbers, the proportion expressing the mutant allele subsequently declined, but whereas those expressing the rumpshaker allele formed a reduced but stable population, the number of jimpy cells fell progressively. The age of decline in the jimpy cells in different regions of the CNS correlated with the temporal sequence of myelination. In compound heterozygotes expressing rumpshaker and jimpy alleles, oligodendrocytes expressing the former predominated and were more abundant than when the rumpshaker and null alleles were in competition. Thus, oligodendrocyte survival is not determined solely by cell intrinsic factors, such as the conformation of the misfolded PLP, but is influenced by neighboring cells, possibly competing for cell survival factors.
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影响因子：
4.2
作者：
GOW, A;FRIEDRICH, VL;LAZZARINI, RA
通讯作者：
LAZZARINI, RA
DOI：
10.1111/j.1365-2990.1991.tb00729.x
发表时间：
1991-08-01
影响因子：
5
作者：
FANARRAGA, ML;GRIFFITHS, IR;KENNEDY, PGE
通讯作者：
KENNEDY, PGE
影响因子：
6.2
作者：
FANARRAGA, ML;GRIFFITHS, IR;BROPHY, PJ
通讯作者：
BROPHY, PJ
影响因子：
30.8
作者：
Gow, A;Lazzarini, RA
通讯作者：
Lazzarini, RA
DOI：
10.1007/bf01217305
发表时间：
1990-04-01
期刊：
JOURNAL OF NEUROCYTOLOGY
影响因子：
--
作者：
GRIFFITHS, IR;SCOTT, I;CATTANACH, BM
通讯作者：
CATTANACH, BM