Marsh R F, Sipe J C, Morse S S, Hanson R P
Lab Invest. 1976 Apr;34(4):381-6.
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.
已发现18个月及以上且死于传染性水貂脑病（TME）的水貂，如果它们是阿留申基因的纯合子，因而表现出常染色体隐性疾病即切迪雅克-东综合征（CH综合征），其大脑的海绵状变性会显著减少。1岁以下的CH水貂以及年轻或年老的非CH水貂具有典型的病变特征，即神经纤维网广泛出现微空泡化。而年老的CH水貂在光学和电子显微镜水平上的海绵状变性均减少，但TME疾病过程中没有其他明显改变。TME病原体的潜伏期、临床症状、星形细胞反应及脑内浓度与非CH水貂所见相当。我们得出结论，海绵状变性是TME中的继发性变化，并推测空泡化可能是溶酶体酶导致神经节苷脂分解代谢增加的结果。
