Strial microvascular pathology and age-associated endocochlear potential decline in NOD congenic mice.
Strial microvascular pathology and age-associated endocochlear potential decline in NOD congenic mice.
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DOI：
10.1016/j.heares.2008.08.001
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发表时间：
2008-10
期刊：
影响因子：
2.8
通讯作者：
Gagnon PM
中科院分区：
文献类型：
--
作者：
Ohlemiller KK;Rice ME;Gagnon PM
NOD/ShiLtJ（以前的NOD/LtJ）近交系小鼠显示多基因自身免疫性疾病，通常用于模拟自身免疫相关的I型糖尿病以及干燥综合征。他们还表现出快速进展的听力损失，部分原因是Cdh 23 ahl和Ahl 2的联合作用。在H2组织相容性基因复合体中携带校正等位基因的同基因NOD. NON-H2 nb 1/LtJ小鼠没有糖尿病和其他明显的自身免疫性疾病体征，但仍表现出快速进行性听力损失。在这里，我们表明，在这些先天性耳蜗病理广泛包括毛细胞和神经元的损失，加上耳蜗内电位（EP）下降，从最初的正常值后2个月的年龄。EP减少通常伴随着血管纹中毛细血管的急剧变性，导致血管变性。同种小鼠的耳蜗蜗轴也具有类似于某些小鼠自身免疫模型中的血管周围包涵体的特征。我们证实NOD. NON-H2 nb 1小鼠耳蜗毛细胞/神经和耳蜗毛细胞病变是独立发生的。虽然感觉细胞损失可能与Cdh 23 ahl和Ahl 2密切相关，但我们观察到的微血管病理学和蜗轴异常可能源于与免疫功能相关的NOD背景上的等位基因。NOD. NON-H2 nb 1小鼠中与听力损失相关的EP下降可以模拟主要由微血管疾病引起的听力损失。这些小鼠中显著的毛细血管损失也可用于研究血管功能不全与血管功能之间的关系。
NOD/ShiLtJ (previously NOD/LtJ) inbred mice show polygenic autoimmune disease and are commonly used to model autoimmune-related Type I diabetes, as well as Sjogren’s syndrome. They also show rapidly progressing hearing loss, partly due to the combined effects of Cdh23ahl and Ahl2. Congenic NOD.NON-H2nb1/LtJ mice, which carry corrective alleles within the H2 histocompatibility gene complex, are free from diabetes and other overt signs of autoimmune disease, but still exhibit rapidly progressive hearing loss. Here we show that cochlear pathology in these congenics broadly includes hair cell and neuronal loss, plus endocochlear potential (EP) decline from initially normal values after 2 months of age. The EP reduction follows often dramatic degeneration of capillaries in stria vascularis, with resulting strial degeneration. The cochlear modiolus in the congenic mice also features perivascular inclusions that resemble those in some mouse autoimmune models. We posit that cochlear hair cell/neural and strial pathology in NOD.NON-H2nb1 mice arise independently. While sensory cell loss may be closely tied to Cdh23ahl and Ahl2, the strial microvascular pathology and modiolar anomalies we observe may arise from alleles on the NOD background related to immune function. Age-associated EP decline in NOD.NON-H2nb1 mice may model forms of strial age-related hearing loss caused principally by microvascular disease. The remarkable strial capillary loss in these mice may also be useful for studying the relation between strial vascular insufficiency and strial function.
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影响因子：
1.4
作者：
JOHNSSON, LG;HAWKINS, JE
通讯作者：
HAWKINS, JE
影响因子：
2.8
作者：
Ohlemiller, Kevin K.;Lett, Jadynn M.;Gagnon, Patricia M.
通讯作者：
Gagnon, Patricia M.
影响因子：
1.7
作者：
McQueen, CT;Baxter, A;Pillsbury, HC
通讯作者：
Pillsbury, HC
DOI：
10.1007/s10162-007-0089-4
发表时间：
2007-12-01
影响因子：
2.4
作者：
Diaz, Rodney C.;Ana, Elena Vazquez;Yamoah, Ebenezer N.
通讯作者：
Yamoah, Ebenezer N.
影响因子：
2.8
作者：
Fujimura, T;Suzuki, H;Doi, Y
通讯作者：
Doi, Y