Transmissible mink encephalopathy. Reduced spongiform degeneration in aged mink of the Chediak-Higashi genotype.
Transmissible mink encephalopathy. Reduced spongiform degeneration in aged mink of the Chediak-Higashi genotype.
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传染性水貂脑病。
DOI：
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发表时间：
1976
期刊：
影响因子：
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通讯作者：
Hanson Rp
中科院分区：
文献类型：
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作者：
Marsh Rf;Sipe Jc;Morse Ss;Hanson Rp
18个月或更大的水貂死于传染性水貂脑病（TME），如果它们是阿留申基因纯合型的，那么它们的大脑海绵状变性明显减少，因此表现出常染色体隐性遗传病，称为Chediak Higashi（CH）综合征。小于1岁的CH貂和年轻或年老的非CH貂具有典型的病变轮廓，神经堆广泛微空泡化。而老年CH水貂减少海绵状变性在光镜和电镜水平，有没有其他明显的改变，在TME疾病的过程。TME试剂的孵育时间、临床体征、星形胶质细胞反应和脑浓度与在非CH水貂中观察到的相当。我们得出结论，海绵状变性是TME的继发性变化，并推测空泡化可能是溶酶体酶引起神经节苷脂catalysts增加的结果。
: Mink which are 18 months or older and are dying of transmissible mink encephalopathy (TME) have been found to have a marked reduction in spongiform degeneration of the brain if they are homozygous for the Aleutian gene and thus exhibit the autosomal recessive disorder known as the Chediak Higashi (CH) syndrome. CH mink younger than 1 year, and young or old non-CH mink have a typical lesion profile with widespread microvacuolation of the neuropile. Whereas aged CH mink have reduced spongiform degeneration at both the light and electron microscopic level, there is no other apparent alteration in the TME disease process. The length of incubation, clinical signs, astrocytic response, and brain concentration of the TME agent are comparable to those seen in non-CH mink. We conclude that spongiform degeneration is a secondary change in TME and speculate that vacuolation may be the result of lysosomal enzymes causing an increase in ganglioside catabolism.