Therapeutic Antibodies Against Prion Diseases From PRNP Mutation Carriers (University of Zurich, NCT02837705)

A completed, single-visit observational case-control study sponsored by the University of Zurich. It collected one blood sample each from people in families with genetic prion disease, comparing carriers of a disease-associated PRNP variant with relatives who do not carry one, to measure natural IgG antibodies against the prion protein. The idea was that late onset in inherited disease might reflect protective natural antibodies that could be copied as therapy. The published result was negative: antibody levels did not differ by mutation status or clinical state.

Status as of 2026-11-01. Please check the study registry for the latest position.

Key facts

  • Sponsor is the University of Zurich with no collaborators listed in the registry; the record names no investigators.
  • Design: observational, case-control, cross-sectional, one blood draw, target duration one day, 213 participants enrolled (actual).
  • Two arms: carriers of a PRNP variant (symptomatic or presymptomatic) and wild-type family members; ages 1 to 99 accepted.
  • Primary outcome is the level of anti-prion protein autoantibodies; no results were posted on the registry.
  • Seven listed sites: UCSF Memory and Aging Center, Medical University Graz, University Medical Center Goettingen, CJD Foundation Israel, Istituto di Ricerche Farmacologiche (Milan), Slovak Medical University (Bratislava) and the Institute of Neuropathology in Zurich.
  • The publication analysed 202 samples (124 carriers, 78 controls from affected families without a variant) with an ELISA for human IgG1 to IgG4 against wild-type human prion protein.
  • Reactivity was found in a subset of both carriers and controls; mutation status and clinical manifestation did not change titers, and titers were independent of codon 129 and autoimmune history.
  • The authors concluded that pathogenic PRNP variants do not notably stimulate anti-PrPC antibody immunity and that such antibodies are not tied to disease onset.
  • Zurich was lead regulatory site, ethics approval came from the Cantonal Ethics Committee of Zurich, and raw data are withheld because of re-identification risk.
  • Stated funders include EPSRC, BBSRC, ERC, NIH and CDC grants, and Prion Alliance funded some sample collection; the authors state funders had no role.

Timeline

  1. 2015Study start (actual).
  2. 2016First registered on ClinicalTrials.gov (first submitted 2016-07-07, posted 2016-07-20).
  3. 2018Primary completion.
  4. 2019Study completion.
  5. 2020Results published online in Neurology (print 2020-10-06).

People

Last checked 1 November 2026

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