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Pedigree of family affected with PRNP Q160X. An autosomal dominant inheritance pattern was noted. The proband's asymptomatic father was a confirmed heterozygous Q160X carrier. The proband's paternal grandfather had severe dysautonomia, peripheral neuropathy, and cognitive decline. The arrow denotes the proband. The asterisks correspond to genetically confirmed PRNP Q160X cases. MM, codon 129 methionine homozygosity; MV, codon 129 heterozygosity; d., death age.
