Filamentous white matter prion protein deposition is a distinctive feature of multiple inherited prion diseases.

Acta neuropathologica communications (2013-11-21)
Lilla Reiniger, Ilaria Mirabile, Ana Lukic, Jonathan Df Wadsworth, Jacqueline M Linehan, Michael Groves, Jessica Lowe, Ronald Druyeh, Peter Rudge, John Collinge, Simon Mead, Sebastian Brandner

Sporadic, inherited and acquired prion diseases show distinct histological patterns of abnormal prion protein (PrP) deposits. Many of the inherited prion diseases show striking histological patterns, which often associate with specific mutations. Most reports have focused on the pattern of PrP deposition in the cortical or cerebellar grey matter. We observed that the subcortical white matter in inherited prion diseases frequently contained filamentous depositions of abnormal PrP, and we have analysed by immunohistochemistry, immunofluorescence and electron microscopy 35 cases of inherited prion disease seen at the UK National Prion Clinic. We report here that filamentous PrP is abundantly deposited in myelinated fibres in inherited prion diseases, in particular in those with N-terminal mutations. It is possible that the presence of filamentous PrP is related to the pathogenesis of inherited forms, which is different from those sporadic and acquired forms.

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Monoclonal Anti-Neurofilament 200 antibody produced in mouse, clone NE14, ascites fluid