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先天性颤抖,鸡的一种遗传性神经缺陷。

Congenital quiver, an inherited neurological defect in the chicken.

作者信息

Smyth J R, Jerszyk M M, Montgomery N

出版信息

J Hered. 1985 Jul-Aug;76(4):263-6. doi: 10.1093/oxfordjournals.jhered.a110089.

Abstract

A new neurological defect in the chicken, congenital quiver (CQ), is characterized by a continuous tremor. At hatching, affected chicks show a rapid tremor, while adult expression varies from a slight quiver to pronounced shaking. Genetic analysis showed the defect to be inherited as an autosomal recessive gene (cq) with complete penetrance. Livability of CQ birds is equivalent to that of their normal sibs. Affected females lay eggs and produce chicks; however, males are essentially sterile. Histologically, their testes show considerable cellular disorganization and a marked reduction in spermatogenic elements. Semen quality evaluations showed CQ males to be significantly inferior in respect to semen volume, sperm concentration, initial motility, and percent normal sperm. Histological examination of CQ brains revealed a number of pathological changes, including extensive degeneration of fiber tracts and neurons across the neuroaxis. There also is a diffuse loss of Purkinje cells in the cerebellar cortex with the remaining cell somas becoming extremely eosinophilic. In addition, many of the blood vessels throughout the white matter showed perivascular cuffing.

摘要

鸡的一种新的神经缺陷,即先天性震颤(CQ),其特征为持续性震颤。在孵化时,受影响的雏鸡表现出快速震颤,而成年鸡的表现则从轻微颤抖到明显摇晃不等。遗传分析表明,该缺陷作为常染色体隐性基因(cq)遗传,具有完全外显率。CQ鸡的存活率与它们正常的同胞相当。受影响的雌性能够产卵并孵化出雏鸡;然而,雄性基本上不育。组织学上,它们的睾丸显示出相当程度的细胞紊乱,生精细胞显著减少。精液质量评估显示,CQ雄性在精液量、精子浓度、初始活力和正常精子百分比方面明显较差。对CQ鸡大脑的组织学检查发现了一些病理变化,包括整个神经轴上纤维束和神经元的广泛退化。小脑皮质中浦肯野细胞也有弥漫性丧失,其余细胞体变得极度嗜酸性。此外,整个白质中的许多血管出现血管周围套袖现象。

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