About this trial
The purpose of this trial is to evaluate safety, tolerability, pharmacokinetics and pharmacodynamic impact of PrP-siRNA in symptomatic prion disease patients.
Eligibility criteria
This trial does not accept healthy volunteersQualifiers
clinically manifested symptoms of prion disease, in the opinion of the investigator;
a diagnosis of probable prion disease according to CDC criteria;
a positive CSF RT-QuIC or PRNP genetic test;
no more than moderate functional impairment as quantified by an MRC-PDRS score ≥15; and
Disqualifiers
pregnancy;
contraindication to LP; or
recent participation in a different prion disease clinical trial.
Trial design
Sequential
Treatments tested in this trial
PrP-siRNA
DrugIntrathecally administered divalent siRNA designed to target the PRNP mRNA. The structure has been published in DOI: 10.1101/2024.12.05.627039
Treatment groups
Trial outcomes
Primary outcomes
Frequency of adverse events
Secondary outcomes
CSF PrP concentration
Prion protein (PrP) concentration in cerebrospinal fluid (CSF), a pharmacodynamic (PD) biomarker for PrP-siRNA activity
CSF PrP concentration
Prion protein (PrP) concentration in cerebrospinal fluid (CSF), a pharmacodynamic (PD) biomarker for PrP-siRNA activity
CSF PrP concentration
Prion protein (PrP) concentration in cerebrospinal fluid (CSF), a pharmacodynamic (PD) biomarker for PrP-siRNA activity
CSF PrP concentration
Prion protein (PrP) concentration in cerebrospinal fluid (CSF), a pharmacodynamic (PD) biomarker for PrP-siRNA activity
Sponsors and contacts
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Broad Institute of MIT and Harvard
Lead sponsor
National Institute of Neurological Disorders and Stroke (NINDS)
Collaborator