The Briard dog: a new animal model of congenital stationary night blindness.
The Briard dog: a new animal model of congenital stationary night blindness.
复制标题
Briard狗：先天性静止性夜盲症的新动物模型。
DOI：
10.1136/bjo.73.9.750
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发表时间：
1989
影响因子：
4.1
通讯作者：
S E Nilsson
中科院分区：
文献类型：
--
作者：
Kristina Narfström;A. Wrigstad;S E Nilsson
在瑞典的一窝Briard犬中观察到先天性静止性夜盲症（CSNB），明显以常染色体隐性方式遗传。9窝中有5窝患夜盲症。不同的临床测试，包括视网膜电图（ERG）的结果进行了比较，发现在四个人类病例的CSNB，其中三个最有可能与常染色体隐性遗传和X连锁遗传。该疾病的先天性和静止性，眼底镜检查正常，可记录但减少的明视闪烁反应是犬和人病例之间发现的一些相似之处。单闪光ERG反应是异常的，在人类以及受影响的Briards。然而，人类病例显示“阴性”ERG，而在Briards中，a波和B波都极度减少，并且仅存在于明视水平。在人类中也描述了类似于这些Briard的病例，其中发现视紫红质浓度和再生是正常的，表明转导机制受到干扰。因此，Briard犬可能成为人类CSNB的有价值的模型。
Congenital stationary night blindness (CSNB), apparently inherited in an autosomal recessive manner, was observed in a litter of Briard dogs in Sweden. Of nine litter mates five had nyctalopia. The results of different clinical tests, including electroretinography (ERG), were compared with the results found in four human cases of CSNB, three of which were most probably associated with autosomal recessive inheritance and one with X-linked inheritance. The congenital and stationary nature of the disease, ophthalmoscopically normal appearing fundi, and recordable but reduced photopic flicker responses were some of the similarities found between canine and human cases. The single-flash ERG response was abnormal in the humans as well as in the affected Briards. However, the human cases showed a "negative' ERG, whereas in the Briards both the a and b waves were extremely reduced and present only at a photopic level. Cases similar to these Briards have been described also in man, where rhodopsin concentration and regeneration were found to be normal, suggesting a disturbed transduction mechanism. It thus appears that the Briard dog may become a valuable model of human CSNB.