TRANSMISSIBLE MINK ENCEPHALOPATHY - REDUCED SPONGIFORM DEGENERATION IN AGED MINK OF CHEDIAK-HIGASHI GENOTYPE

  • 1 January 1976
    • journal article
    • research article
    • Vol. 34  (4) , 381-386
Abstract
Mink .gtoreq. 18 mo. old, dying of transmissible mink encephalopathy (TME), 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 < 1 yr old 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 the light microscopic and EM 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. Spongiform degeneration is apparently a secondary change in TME; vacuolation may be the result of lysosomal enzymes causing an increase in ganglioside catabolism.