Decline in brainstem auditory-evoked potentials coincides with loss of spiral ganglion cells in arylsulfatase A-deficient mice
Decline in brainstem auditory-evoked potentials coincides with loss of spiral ganglion cells in arylsulfatase A-deficient mice
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DOI：
10.1016/s0006-8993(99)02085-5
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发表时间：
1999-11-20
期刊：
影响因子：
2.9
通讯作者：
De Deyn, PP
中科院分区：
文献类型：
--
作者：
D'Hooge, R;Coenen, R;De Deyn, PP
芳基硫酸酯酶A（阿萨）缺陷型小鼠构成了遗传性溶酶体贮积病，异染性脑白质营养不良（MLD）的动物模型。脑干诱发电位（BAEP）记录在对照组和ASA缺陷小鼠的3，6，9和12个月。BAEP在所有年龄的对照小鼠中均被诱发，但在9个月和12个月的阿萨（- / -）小鼠中完全不存在。6月龄阿萨（- / -）小鼠BAEP波形明显延迟。组织学检查和形态计量学分析表明，阿萨（- / -）小鼠BAEP的下降被螺旋神经节细胞数量的减少所掩盖。(C)1999 Elsevier Science B. V.保留所有权利。
Arylsulfatase A (ASA)-deficient mice constitute an animal model for the inherited lysosomal storage disease, metachromatic leukodystrophy (MLD). Brainstem auditory-evoked potentials (BAEPs) were recorded in control and ASA-deficient mice of 3, 6, 9 and 12 months. BAEPs were evoked in control mice of all ages studied, but were completely absent in ASA (- / -) mice of 9 and 12 months. A significant delay in the wave pattern was noted in 6-month-old ASA (- / -) mice, Histological examination and morphometric analysis showed that the decline of BAEPs in ASA (- / -) mice was paralleled by a decrease in spiral ganglion cell numbers. (C) 1999 Elsevier Science B.V. All rights reserved.