A novel behavioral method to detect motoneuron disease in Wobbler mice aged three to seven days old
A novel behavioral method to detect motoneuron disease in Wobbler mice aged three to seven days old
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DOI：
10.1016/s0006-8993(98)01033-6
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发表时间：
1998-12-07
期刊：
影响因子：
2.9
通讯作者：
Vacca-Galloway, LL
中科院分区：
文献类型：
--
作者：
Bose, P;Fielding, R;Vacca-Galloway, LL
Wobbler小鼠具有遗传性常染色体隐性形式的运动神经元疾病。最典型的异常是运动神经元的变性，主要在颈脊髓和脑干颅运动核。潜在的病理学表现为仅在断奶时（3周龄）才能确信地检测到的症状。我们现在报告一种新的方法，旨在确定前驱Wobbler小鼠的行为和统计方法。我们测量了体重、翻正反射（RR）和性别，以检查这些参数是否对3周龄前的疾病状态有影响。使用总共341只NFR/wr品系幼仔，我们发现RR与出生后第3天至第7天的摇摆病状态之间存在强相关性（p < 0.0001），并实现了97%以上的摇摆病正确分类。因此，RR的测量允许以最小的误差早期检测受影响的Wobbler（wr/wr）小鼠。这种方法已被用于在我们的实验室的免疫细胞化学研究，显示早期发芽的免疫反应性5-羟色胺和肽能纤维在颈脊髓腹角出生后7和12天分别。Wobbler小鼠的早期检测因此促进了对运动神经元疾病发病机制的重要新认识。我们现在可以检查潜在的治疗方法，这些方法可能比在有症状的断奶仔猪中给药更有效（工作正在进行中）。(C)1998年由Elsevier Science B. V.出版，版权所有。
The Wobbler mouse possesses an inherited autosomal recessive form of motoneuron disease. The most characteristic abnormality is the degeneration of motoneurons, mostly in the cervical spinal cord, and in the brain stem cranial motor nuclei. The underlying pathology shows up as symptoms that are only detectable confidently around the time of weaning (age 3 weeks). We now report a new method designed to identify presymptomatic Wobbler mice by behavioral and statistical approaches. We measured body weight, righting reflex (RR) and gender to examine whether these parameters have an impact on the status of the disease before age 3 weeks. Using a total of 341 NFR/wr strain pups, we found a strong association between RR and the Wobbler disease status (p < 0.0001) between postnatal days 3 to 7, and achieved greater than 97% correct classification of Wobblers. Therefore the measurement of RR allows the early detection of the affected Wobbler (wr/wr) mice with a minimum of error. This method has been used in our laboratory for immunocytochemical studies that show the early sprouting of immunoreactive serotonin and peptidergic fibers in the cervical spinal ventral horn by postnatal days 7 and 12 respectively. The early detection of Wobbler mice thus facilitates significant new understanding regarding the pathogenesis of motoneuron disease. We can now examine potentially therapeutic approaches which may be more effective than when administered in the symptomatic weanlings (work in progress). (C) 1998 Published by Elsevier Science B.V. All rights reserved.