Conformation Specific Antibodies and Diagnosis of Prion Diseases

Authors: E. Dvořáková;  K. Holada
Authors‘ workplace: Ústav imunologie a mikrobiologie, 1. LF UK a VFN v Praze
Published in: Cesk Slov Neurol N 2012; 75/108(3): 283-290
Category: Review Article


Transmissible spongiform encephalopathies, or prion diseases, are fatal neurodegenerative disorders with long incubation period and short clinical phase. The diagnosis is usually confirmed from post mortem detection of prions in the brain tissue. Most diagnostic methods are based on treatment with Proteinase K that cleaves out physiological proteins and makes the resistant form of pathological prion protein detectable with anti-prion antibodies. Over the last five years, there is a growing evidence of prionopathies caused by protease-sensitive prions escaping detection with the standard diagnostic methods. Conformation-specific monoclonal antibodies to the pathological prion proteins could overcome the problem simply by detecting the pathological conformation of both prion isoforms without the need for proteolysis. Our review summarizes available information on conformation-specific prion antibodies and discusses their use in the diagnosis of prion diseases.

Key words:
transmissible spongiform encephalopathy – prion protein – conformation-specific antibodies – Creutzfeldt-Jakob disease


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