THE PATHOGENESIS OF GLOBOID-CELL LEUCODYSTROPHY IN PERIPHERAL-NERVE OF THE MOUSE MUTANT TWITCHER
THE PATHOGENESIS OF GLOBOID-CELL LEUCODYSTROPHY IN PERIPHERAL-NERVE OF THE MOUSE MUTANT TWITCHER
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DOI：
10.1016/0022-510x(82)90127-7
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发表时间：
1982-01-01
影响因子：
4.4
通讯作者：
DEARANDA, FT
中科院分区：
文献类型：
--
作者：
JACOBS, JM;SCARAVILLI, F;DEARANDA, FT
抽搐鼠受到一种疾病的影响，其病理特征与人类球状细胞白质营养不良（克拉伯病）相似：它也有相同的酶缺乏症。研究了周围神经病变的进展。髓鞘发育正常，直到第15天左右，髓鞘形成率下降。脱髓鞘，在临床症状出现前10 -11天，首先影响到旁腺区域。几天后巨噬细胞和雪旺细胞可见蟹黄型包涵体。随着年龄的增长，脱髓鞘变得广泛，影响各种大小的纤维，轴突迅速重新脱髓鞘。轴突不参与其中，但定量研究表明，它们仍然比正常情况小。将抽搐神经的变化与人类克拉伯氏病的神经病变进行比较。
The twitcher mouse is affected by a disease with pathological features resembling those of human globoid cell leukodystrophy (Krabbe''s disease): it also has an identical enzyme deficiency. The progress of the disease was studied in peripheral nerves. Myelin sheaths develop normally until about the 15th day when the rate of myelination declines. Demyelination, first affecting paranodal regions is seen from the 10th-11th day before clinical signs appear. Krabbe-type inclusions are seen in macrophages and Schwann cells a few days later. Demyelination becomes extensive with increasing age, affecting fibers of all sizes, and axons rapidly remyelinate. Axons are not involved but quantitative studies show that they remain smaller than normal. Changes in twitcher nerves are compared to those in the neuropathy in human Krabbe''s disease.