RUMPSHAKER - AN X-LINKED MUTATION CAUSING HYPOMYELINATION - DEVELOPMENTAL DIFFERENCES IN MYELINATION AND GLIAL-CELLS BETWEEN THE OPTIC-NERVE AND SPINAL-CORD
RUMPSHAKER - AN X-LINKED MUTATION CAUSING HYPOMYELINATION - DEVELOPMENTAL DIFFERENCES IN MYELINATION AND GLIAL-CELLS BETWEEN THE OPTIC-NERVE AND SPINAL-CORD
复制标题
DOI：
10.1002/glia.440050302
复制
发表时间：
1992-01-01
期刊：
影响因子：
6.2
通讯作者：
BROPHY, PJ
中科院分区：
文献类型：
--
作者：
FANARRAGA, ML;GRIFFITHS, IR;BROPHY, PJ
X连锁突变rumpshaker（rsh）可能是jimpy（jp）的等位基因，导致小鼠中枢神经系统髓鞘形成不足。本研究探讨了视神经和脊髓的形态学、胶质细胞和髓鞘蛋白的免疫染色的发育表达。视神经包含不同数量的无髓鞘和有髓鞘纤维。大多数这样的鞘是正常的厚度，而在脊髓中，大多数轴突与不成比例的薄鞘有关，其在发育期间厚度变化很小。在视神经中，神经胶质细胞数量在髓鞘形成的早期和高峰期在突变体中升高，但随后在成人中略低于正常。相比之下，脊髓中胶质细胞的数量在16日龄后持续升高。总胶质细胞的大多数改变是由于少突胶质细胞群的相应变化。髓鞘碱性蛋白（MBP）和蛋白脂质蛋白（PLP）和DM-20共有的C-末端的免疫染色强度有所降低，PLP特异性肽的免疫染色强度显著降低。胶质细胞酸性蛋白（GFAP）在rsh中增加。在rsh中，视神经和脊髓之间髓鞘形成的某些差异可能与两个部位轴突直径的差异有关，因为在髓鞘形成时有足够数量的少突胶质细胞。然而，突变对大脑和脊髓中细胞发育的影响可能不同。免疫染色表明髓鞘中PLP明显缺乏，但表明DM-20水平可能相对正常。rsh与jp和其他X-连锁髓鞘突变体有几个主要差异，特别是在少突胶质细胞数量方面，将有助于阐明PLP基因在影响少突胶质细胞分化和存活中的作用。
The X-linked mutation rumpshaker (rsh), which is probably an allele of jimpy (jp), causes hypomyelination in the CNS of mice. This study examines the developmental expression of the morphology, glial cells, and immunostaining of myelin proteins in the optic nerve and spinal cord. The optic nerve contains varying numbers of amyelinated and myelinated fibres. The majority of such sheaths are of normal thickness whereas in the spinal cord most axons are associated with a disproportionately thin sheath which changes little in thickness during development. In the optic nerve glial cell numbers are elevated in mutants during early and peak myelination but then fall slightly below normal in adults. In contrast, the number of glial cells is consistently elevated after 16 days of age in the spinal cord. The majority of the alterations to total glial cells are due to corresponding changes in the oligodendrocyte population. Immunostaining intensity is somewhat reduced for myelin basic protein (MBP) and the C-terminal common to proteolipid protein (PLP) and DM-20 and profoundly decreased for the PLP-specific peptide. Glial fibrillary acidic protein (GFAP) is increased in rsh. It is probable that some of the variation in myelination between optic nerve and cord in rsh is related to the difference in axon diameter in the two locations, as there are adequate numbers of oligodendrocytes at the time of myelination. However, the effect of the mutation on cell development in the brain and the spinal cord may be different. The immunostaining indicates a marked deficiency in PLP in myelin but suggests that DM-20 levels may be relatively normal. rsh shows several major differences from jp and other X-linked myelin mutants, particularly in relation to oligodendrocyte numbers, and will be useful to elucidate the role of the PLP gene in influencing oligodendrocyte differentiation and survival.