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髓鞘形成不足的突变小鼠:jpmsd的描述及其在当前遗传背景下与jp和qk的比较。

Hypomyelinated mutant mice: description of jpmsd and comparison with jp and qk on their present genetic backgrounds.

作者信息

Billings-Gagliardi S, Adcock L H, Wolf M K

出版信息

Brain Res. 1980 Aug 4;194(2):325-38. doi: 10.1016/0006-8993(80)91215-9.

Abstract

Hypomyelinated mutant mice are valuable natural animal systems for analysis of CNS myelin development, chemistry and diseases. One mutant, jpmsd, has never received thorough morphological description. We here describe the detailed morphology of jpmsd, compare it with well-studied jp and qk on their present genetic backgrounds, and discuss the genetic histories of all 3 mutants. Region for region, jpmsd has twice as much myelin as jp, but 1/2--1/5 as much as qk. Both jp and jpmsd have scarce oligodendrocytes, rare nodes of Ranvier, clustering of myelin segments, abnormal lipid-filled cells, frequent degenerating cells, and rare distorted myelin profiles. In contrast, qk has abnormally numerous oligodendrocytes, frequent nodes of Ranvier, no obvious myelin clustering, no lipid-filled cells, rare degenerating cells, and frequent abnormal or distorted myelin profiles. jp and jpmsd are quantitatively different, but qualitatively similar. Since its origin, the jpmsd disease has inadvertently been ameliorated by transferring the mutation to a different background. Persistent differences in the remainder of the genome might account for all remaining apparent differences between jp and jpmsd. In contrast, qk is totally dissimilar in morphology and presumably in pathophysiology.

摘要

髓鞘形成不足的突变小鼠是用于分析中枢神经系统髓鞘发育、化学组成及疾病的宝贵天然动物模型。一种名为jpmsd的突变小鼠,此前从未得到过全面的形态学描述。我们在此详细描述了jpmsd的形态,将其与在当前遗传背景下已得到充分研究的jp和qk进行比较,并探讨了这三种突变小鼠的遗传史。逐区域来看,jpmsd的髓鞘量是jp的两倍,但仅为qk的1/2至1/5。jp和jpmsd都存在少突胶质细胞稀少、郎飞结罕见、髓鞘节段聚集、脂质填充细胞异常、细胞频繁退化以及髓鞘形态罕见扭曲等情况。相比之下,qk具有异常大量的少突胶质细胞、频繁出现的郎飞结、无明显的髓鞘聚集、无脂质填充细胞、罕见的退化细胞以及频繁出现的异常或扭曲的髓鞘形态。jp和jpmsd在数量上存在差异,但在质量上相似。自其起源以来,通过将该突变转移到不同背景中,jpmsd疾病无意中得到了改善。基因组其余部分的持续差异可能解释了jp和jpmsd之间所有剩余的明显差异。相比之下,qk在形态上以及推测在病理生理学上完全不同。

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