Mutation of the Prion Protein in Libyan Jews with Creutzfeldt–Jakob Disease

Abstract

Creutzfeldt—Jakob disease is a transmissible neurodegenerative disorder that occurs more than 100 times more frequently among Libyan Jews than in the worldwide population. We examined 11 patients with the disease — 10 Libyan Jews from Israel and 1 Libyan Jew from Italy — to determine whether abnormalities of the prion protein could be detected in them. Abnormal forms of this host-encoded protein are the predominant if not sole components of the transmissible agent that causes the disease.