Limited transmission of V180I genetic Creutzfeldt-Jakob disease in knock-in mice models

TL;DR

This study shows that the V180I mutation in Creutzfeldt-Jakob disease is less likely to spread in mice models, which could help reduce risks in medical settings.

Contribution

The study provides new evidence on the limited transmissibility of the V180I prion in humanized mice.

Findings

Tissue from V180I genetic CJD cases shows significantly limited transmissibility in humanized knock-in mice.

The results suggest a lower risk of iatrogenic transmission associated with the V180I mutation.

Abstract

Differentiating Creutzfeldt-Jakob disease (CJD) with the V180I mutation from other types of dementia is extremely difficult. Additionally, its differentiation is sometimes determined after performing neurosurgery, which is associated with a high risk of V180I prion contamination; however, the infectivity of the V180I prion has not been properly investigated. Especially in East Asia, this issue must be addressed to respond effectively to accidental contamination that leads to iatrogenic CJD. The results of our transmission experiments involving various humanized knock-in mice clearly indicate that the transmissibility of tissue from V180I genetic CJD cases is significantly limited.

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