How can you differentiate mitochondrial vs. prion-based disease?
There are very few types of inherited prion diseases, and these would follow a normal dominant trait pattern of inheritance. For autosomal, 50-100% of children of an affected parent (mom or dad) would be affected, depending on genotypes of parents and children. This would look different from maternal inheritance, in which 100% of children of an affected mother are affected, while an affected father would not pass on the trait. This would also look different from infection, which is the other way to develop a prion disease. To distinguish between the conditions in yeast, you can mutate hsp104 or treat with GuHCl (protein denaturant) to cure a prion state but not the mitochondrial defect, or you can sequence mtDNA and look for deletions. IV.
