A quantitative survey of gravity receptor function in mutant mouse strains
A quantitative survey of gravity receptor function in mutant mouse strains
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DOI：
10.1007/s10162-005-0009-1
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发表时间：
2005-12-01
影响因子：
2.4
通讯作者：
Jones, TA
中科院分区：
文献类型：
--
作者：
Jones, SM;Johnson, KR;Jones, TA
本研究的目的是利用线性前庭诱发电位(VsEP)识别小鼠的前庭缺陷。分析了24个已知基因突变的菌株和6个近交系背景菌株的VsEP阈值、峰潜伏期和峰幅度，并对每个菌株进行了描述性统计。将突变纯合子的反应参数与杂合子和/或背景对照进行比较，并将所有菌株的平均值与标准范围进行对比。以下隐性突变纯合子在受试年龄无VsEP：ESPN(Je)、Atp2b2(Dfw-2j)、Spnb4(qv-lnd2j)、Spnb(qv-3j)、MYO7A(SH1)、Tmie(Sr)、Myo6(Sv)、Jc、PCDH15(av-j)、PCDH15(av-2j)、PCDH15(av-3j)、CDH23(v-2j)、SANS(Js)、hr、KCNE1(R)和Pou3f4(Del)。这些结果表明这些纯合子存在严重的重力感受器缺陷，这与许多这些菌株的结构性缺陷是一致的。Catna 2(CDF)、GRID2(Ho4j)、Wnt1(Sw)、Qk和MBP(Si)株纯合子和GRID2(Lc)株杂合子有可测量的VsEP，但有一个或多个反应参数与各自的对照组(杂合子或背景株)不同或超出正常范围。例如，QK和MBP(SHI)纯合子，表现出与这些菌株的异常髓鞘一致的显著延长的潜伏期。潜伏期延长可能提示神经传导障碍；阈值升高提示敏感度降低，幅度降低可能提示神经同步性降低。一个突变，Otx1(Jv)，所有的VsEP反应参数都在正常范围内--这是一个预期的发现，因为Otx1(Jv)的异常可能局限于外半规管。有趣的是，一些杂合子群体在一个或多个VsEP反应参数中也表现出异常，这表明尽管前庭功能障碍没有那么严重，但在一些杂合子动物中可能存在。
The purpose of this research was to identify vestibular deficits in mice using linear vestibular evoked potentials (VsEPs). VsEP thresholds, peak latencies, and peak amplitudes from 24 strains with known genetic mutations and 6 inbred background strains were analyzed and descriptive statistics generated for each strain. Response parameters from mutant homozygotes were compared with heterozygote and/or background controls and all strain averages were contrasted to normative ranges. Homozygotes of the following recessive mutations had absent VsEPs at the ages tested: Espn(je), Atp2b2(dfw-2j), Spnb4(qv-lnd2j), Spnb(qv-3j), Myo7a(sh1), Tmie(sr), Myo6(sv), jc, Pcdh15(av-j), Pcdh15(av-2j), Pcdh15(av-3j), Cdh23(v-2j), Sans(js), hr, Kcne1(pkr), and Pou3f4(del). These results suggest profound gravity receptor deficits for these homozygotes, which is consistent with the structural deficits that have been documented for many of these strains. Homozygotes of Catna2(cdf) Grid2(ho4j), Wnt1(sw), qk, and Mbp(shi) strains and heterozygotes of Grid2(lc) had measurable VsEPs but one or more response parameters differed from the respective control group (heterozygote or background strain) or were outside normal ranges. For example, qk and Mbp(shi) homozygote, showed significantly prolonged latencies consistent with the abnormal myelin that has been described for these strains. Prolonged latencies may suggest deficits in neural conduction; elevated thresholds suggest reduced sensitivity, and reduced amplitudes may be suggestive for reduced neural synchrony. One Mutation, Otx1(jv), had all VsEP response parameters within normal limits-an expected finding because the abnormality in Otx1(jv) is presumably restricted to the lateral semicircular canal. Interestingly, some heterozygote groups also showed abnormalities in one or more VsEP response parameters, suggesting that vestibular dysfunction, although less severe, may be present in some heterozygous animals.