Behavioral and pathological characterization of YG8R transgenic mouse models of Friedreich ataxia
10.19845/j.cnki.zfysjjbzz.2023.0079
- VernacularTitle:弗里德赖希共济失调YG8R转基因小鼠的运动行为学及病理变化比较
- Author:
Xiaojing ZHANG
1
;
Wenqian OU
1
Author Information
1. Departmnet of Pharmacy,The Sixth Affiliated Hospital of Guangzhou Medical University,Qingyuan People Hospital,Qingyuan 511518,China
- Publication Type:Journal Article
- Keywords:
Friedreich ataxia;
YG8R mice;
Behavioral characterization;
Frataxin
- From:
Journal of Apoplexy and Nervous Diseases
2023;40(4):327-331
- CountryChina
- Language:Chinese
-
Abstract:
Objective This study aimed to compare the motor behavior and pathological changes of YG8R transgenic mouse models of Friedreich ataxia (FRDA). Methods The movement behavior changes between YG8R mice and littermate wild-type (WT) mice were evaluated by the accelerated rotarod test,open field test,grip strength test and gait analysis. Real-time fluorescence-based quantitative PCR (qRT-PCR) was used to detect the Frataxin mRNA in the dorsal root ganglia (DRG) and cerebellum. Immunohistochemical staining was used to evaluate neuronal death in the mouse spinal cord tissue. Results Compared with WT mice,the time-to-fall off on an accelerating rotarod,movement distance,and grip strength of YG8R mice were significantly decreased (P<0.05) from 4 to 6 months. Compared to WT mice,YG8R mice had significantly decreased left and right hindlimb step lengths (P<0.05),increased forelimb and hindlimb step widths (P<0.01),decreased right hind limb swing time (P<0.01),and increased the touchdown time of the left and right hind limbs (P<0.05). The number of neurons in the spinal cord of YG8R mice was significantly decreased (P<0.01). The qRT-PCR results showed that frataxin mRNA levels in the spinal cord and DRG of YG8R mice were significantly decreased compared with the WT group (P<0.0001). Conclusion This study further provides detailed motor characteristics and pathological changes of the YG8R transgenic mouse models of FRDA based on GAA repeat expansion,which will be helpful for follow-up studies on the disease mechanism and treatment of FRDA.
- Full text:2024061719384227299Behavioral and pathological characterization of YG8R transgenic mouse models of Friedreich ataxia.pdf
