The Generative Brain
CIC bioGUNE Prion Research Lab (Basque Research and Technology Alliance) logo

CIC bioGUNE Prion Research Lab (Basque Research and Technology Alliance)

CIC bioGUNE in Derio, Spain, home of Joaquin Castilla's Prion Research Lab, which holds a 2024 CJD Foundation grant for dominant-negative protein gene therapy and detects prions in urine.

Key facts

  • Ikerbasque Research Professor and group leader of the Prion Research Lab; prion research since 1998; collaborates with more than 20 teams; reported a homozygous R136S PRNP mutation causing inherited early-onset prion disease.
  • 2024 grants: Kong (NPDPSC) ADAM8-based gene therapy; Schmitz (Goettingen) tear fluid diagnostic; Schmitt-Ulms (Toronto) small brain-penetrant molecule that reduces PrPC and mouse models of spontaneous prion emergence; Castilla (CIC bioGUNE) dominant-negative protein-based gene therapy; Aguzzi and Caredio (Zurich) GPNMB as therapeutic target; organoids from sporadic CJD donors (RML postdoc).
  • Castilla has led the Prion Research Lab at CIC bioGUNE since 2009 as Ikerbasque Research Professor, with research lines in dominant-negative gene therapy and prion detection in urine and more than 20 collaborations.
  • The lab's 2024 grant final report (December 2025) screened more than 900 species and 424 recombinant PrP variants and reported mouse survival extensions of 35 to over 100 percent in two prion models.
  • A first-year report recorded toxicity at unexpectedly high expression of the delivered PrP.
  • A February 2026 preprint with Castilla as co-author shows G127V PrP blocks prion infection across strains in cell culture.

Timeline

  1. 2009Prion Research Lab founded.
  2. 2024Grant awarded.
  3. 2025Final grant report posted.
  4. 2026G127V dominant-negative preprint.
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