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首页> 外文期刊>Life sciences >Neurodevelopmental and cognitive behavior of glutaryl-CoA dehydrogenase deficient knockout mice
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Neurodevelopmental and cognitive behavior of glutaryl-CoA dehydrogenase deficient knockout mice

机译：戊二酰辅酶A脱氢酶缺陷型敲除小鼠的神经发育和认知行为

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Aims: The establishment of a genetic knockout murine model of glutaric acidemia type I (GAI) with complete loss of glutaryl-CoA dehydrogenase (GCDH) activity has been used to investigate the pathological mechanisms underlying neurological symptoms in this disorder. However, very little has been reported on the neurobehavior of GCDH deficient mice (Gcdh-/-). Main methods: In the present study we evaluated physical (body and weight gain) and neuromotor development (appearance of coat, upper incisor eruption, eye-opening day, motor coordination, muscular strength and climbing), as well as cognitive behavior (inhibitory avoidance) in Gcdh-/-, as compared to wild type (WT) mice. Key findings: We found that Gcdh-/- mice did not differ in body and weight gain, appearance of coat, upper incisor eruption, motor coordination and muscular strength, but had a significant delayed eye opening, implying a mild impairment of neurodevelopment in these animals. Furthermore, the climbing behavior was significantly higher in Gcdh-/- as compared to WT mice, suggesting an altered dopaminergic function. Finally, Gcdh-/- mice presented a deficit of short- and long-term memories in the inhibitory avoidance task. Significance: Although it is difficult to extrapolate the present findings to the human condition, our present data are particularly interesting in view of the psychomotor/mental delay that occurs in a significant number of GAI patients with no previous history of acute encephalopathy with striatum destruction. Strict and early treatment possibly associated with novel therapies seems therefore important to prevent learning/memory disabilities in GAI patients.
机译：目的：建立具有完全丧失戊二酰辅酶A脱氢酶（GCDH）活性的I型戊二酸血症（GAI）基因敲除小鼠模型，已用于研究该疾病神经系统症状的病理机制。但是，关于GCDH缺陷小鼠（Gcdh-/-）的神经行为的报道很少。主要方法：在本研究中，我们评估了身体（体重和体重增加）和神经运动的发展（大衣的外观，上门牙的萌出，睁眼的日子，运动的协调性，肌肉力量和攀爬）以及认知行为（抑制性避免）与野生型（WT）小鼠相比）。关键发现：我们发现Gcdh-/-小鼠的体重和体重增加，被毛外观，上门牙萌出，运动协调和肌肉力量没有差异，但是眼球张开明显延迟，这暗示这些动物的神经发育受到轻度损害动物。此外，与野生型小鼠相比，Gcdh-/-的攀爬行为明显更高，表明多巴胺能功能发生了改变。最后，Gcdh-/-小鼠在抑制回避任务中表现出短期和长期记忆的缺陷。启示：尽管很难将目前的发现推论到人类的病情中，但是鉴于许多先前没有急性脑病伴有纹状体破坏史的GAI患者发生精神运动/心理延迟，我们目前的数据尤其有趣。因此，可能与新颖疗法相关的严格和早期治疗对于预防GAI患者的学习/记忆障碍似乎很重要。

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