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.jpg): Failed to open stream: No such file or directory in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 117 Acta+Neuropathol+Commun
2013 ; 1
(1
): 8
Nephropedia Template TP
gab.com Text
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English Wikipedia
Filamentous white matter prion protein deposition is a distinctive feature of
multiple inherited prion diseases
#MMPMID24252267
Reiniger L
; Mirabile I
; Lukic A
; Wadsworth JD
; Linehan JM
; Groves M
; Lowe J
; Druyeh R
; Rudge P
; Collinge J
; Mead S
; Brandner S
Acta Neuropathol Commun
2013[May]; 1
(1
): 8
PMID24252267
show ga
BACKGROUND: 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. RESULTS: 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. CONCLUSIONS: 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.