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MM2-thalamic Creutzfeldt-Jakob disease: neuropathological, biochemical and transmission studies identify a

Fabio Moda1, Silvia Suardi, Giuseppe Di Fede

  • 1Fondazione IRCCS Istituto Neurologico Carlo Besta, Milan, Italy.

Brain Pathology (Zurich, Switzerland)
|February 1, 2012
PubMed

Insights

Creutzfeldt-Jakob disease (CJD) subtypes are linked to prion protein (PrPSc) types and PRNP gene variations. MM2-thalamic CJD, unlike the cortical form, transmits to mice, indicating a distinct prion strain.

Area of Science:

  • Neuroscience
  • Prion Diseases
  • Molecular Biology

Background:

  • Creutzfeldt-Jakob disease (CJD) classification relies on protease-resistant prion protein (PrPSc) size and PRNP codon 129 polymorphism.
  • Sporadic CJD (sCJD) subtypes correlate with distinct clinical and pathological features.
  • MM2-thalamic CJD is a rare subtype, previously grouped with sporadic fatal insomnia.

Observation:

  • Two young patients presented with MM2-thalamic CJD.
  • Neuropathology revealed diffuse PrP immunoreactivity in the cortex and severe thalamic/olivary nucleus damage.
  • Western blot confirmed type 2A PrPSc in affected individuals.

Findings:

  • Transgenic mice expressing 129MM human PrP successfully transmitted MM2-thalamic sCJD.
  • MM2-cortical sCJD did not transmit to the same mouse model.
  • Affected mice exhibited unprecedented type 2A PrPSc deposition, distinct from other sCJD subtypes.

Implications:

  • MM2-thalamic sCJD represents a unique prion strain, separable from MM2-cortical sCJD.
  • This finding refines the understanding of CJD prion diversity and classification.
  • Further research into prion strain characteristics is crucial for CJD diagnosis and therapeutic strategies.

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