Comprehensive and differential long-term characterization of the alpha-galactosidase A deficient mouse model of Fabry disease focusing on the sensory system and pain development

Nurcan ü?eyler; Lydia Biko; Dorothea Hose; Lukas Hofmann; Claudia Sommer

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Comprehensive and differential long-term characterization of the alpha-galactosidase A deficient mouse model of Fabry disease focusing on the sensory system and pain development

机译：α-半乳糖苷酶的全面和差异的长期表征Fabry疾病的缺陷小鼠模型，着眼于感觉系统和疼痛发展

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Fabry disease is an X-linked lysosomal storage disorder due to impaired activity of alpha-galactosidase A with intracellular accumulation of globotriaosylceramide. Associated small fiber pathology leads to characteristic pain in Fabry disease. We systematically assessed sensory system, physical activity, metabolic parameters, and morphology of male and female mice with alpha-galactosidase A deficiency (Fabry ko) from 2 to 27 months of age and compared results with those of age- and gender-matched wild-type littermates of C57Bl/6J background. From the age of two months, male and female Fabry mice showed mechanical hypersensitivity (p?p?p?p?p?p?p?p?p?p?p?

机译：法布里病是一种X连锁的溶酶体贮积病，是由于α-半乳糖苷酶A的活性受损以及球果糖基神经酰胺在细胞内积累所致。相关的小纤维病理学导致法布里病的特征性疼痛。我们系统地评估了2至27个月大的患有α-半乳糖苷酶A缺乏症（Fabry ko）的雄性和雌性小鼠的感觉系统，体力活动，代谢参数和形态，并将结果与年龄和性别相匹配的野生动物进行了比较键入C57Bl / 6J背景的同窝幼仔。从两个月大的年龄开始，雄性和雌性Fabry小鼠在一周的跑步机实验中显示出机械性超敏反应（p > p > p> p> p > p > p > p > p > <0.001）野生同窝仔。同样，法布里（Fabry）小鼠表现出自发的止痛行为，并发展出类似于法布里（Fabry）病患者的口面部畸形。患有α-半乳糖苷酶A缺乏症的小鼠表现出年龄依赖性和明显的感觉系统缺陷。缺乏α-半乳糖苷酶A的小鼠似乎可以模拟人法布里疾病，并且在研究法布里相关疼痛的病理生理学时可能会有所帮助。

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《Molecular pain》 |2016年第2010期|共页
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Nurcan ü?eyler; Lydia Biko; Dorothea Hose; Lukas Hofmann; Claudia Sommer;
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1. Transgenic mouse expressing human mutant alpha-galactosidase A in an endogenous enzyme deficient background: a biochemical animal model for studying active-site specific chaperone therapy for Fabry disease [J] . Ishii S, Yoshioka H, Mannen K, Biochimica et biophysica acta. Molecular basis of disease: BBA . 2004,第3期

机译：在内源性酶缺乏背景下表达人类突变体α-半乳糖苷酶A的转基因小鼠：用于研究法布里病活性位点特异性伴侣疗法的生化动物模型
2. The alpha-galactosidase A-deficient rat: Characterization of a new animal model of Fabry disease [J] . Miller James J., Aoki Kazuhiro, Tiemeyer Michael, Molecular genetics and metabolism . 2016,第2期

机译：α-半乳糖苷酶A缺陷型大鼠：法布里病新动物模型的表征
3. Progressive hearing loss in the alpha-galactosidase A deficient rat model of Fabry disease [J] . Molecular genetics and metabolism . 2020,第2期

机译：α-半乳糖苷酶的渐进式听力损失缺乏法布里病的大鼠模型
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机译：用啮齿动物和人成人原发性肝细胞的患病小鼠肝脏的重新迁移，upA / Rag-2小鼠模型中移植肝细胞的长期存活
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机译：慢性消耗性疾病转基因小鼠模型的开发和表征：鹿和麋鹿的病毒病。
6. Comprehensive and differential long-term characterization of the alpha-galactosidase A deficient mouse model of Fabry disease focusing on the sensory system and pain development [O] . Nurcan Üçeyler, Lydia Biko, Dorothea Hose, 2016

机译：α-半乳糖苷酶的全面和差异的长期表征Fabry疾病的缺陷小鼠模型着眼于感觉系统和疼痛发展
7. Comprehensive and differential long-term characterization of the alpha-galactosidase A deficient mouse model of Fabry disease focusing on the sensory system and pain development [O] . Üçeyler, Nurcan, Biko, Lydia, Hose, Dorothea, 2017

机译：α-半乳糖苷酶的全面和差异的长期表征Fabry疾病的缺陷小鼠模型，着眼于感觉系统和疼痛发展

Comprehensive and differential long-term characterization of the alpha-galactosidase A deficient mouse model of Fabry disease focusing on the sensory system and pain development

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