CJD is one of the Transmissible Spongiform Encephalopathies (TSE) that affect humans.

TSEs are degenerative disorders of the central nervous system (CNS) and brain that lead to motor dysfunction, dementia, and death.

The exact mechanisms for transmission are yet unknown, however normal prion protein, PrP or PrPc (a ubiquitously expressed protein) undergoes conversion to an abnormal prion protein termed PrPsc.

PrPsc accumulates in the brain and amyloid like aggregates are visible upon histological analysis. These aggregates are associated with the appearance of large patterns of vacuolation. The vacuolation indicates neuronal cell death and the pattern makes the tissue resemble a sponge like appearance.

TSE work is primarily focused to provide reference materials via the CJD Resource Centre and research and development working together with industrial and research partners for the evaluation of assays and new reagents

CJD Resource Centre