PMID- 12172919
OWN - NLM
STAT- MEDLINE
DCOM- 20021017
LR  - 20071109
IS  - 0001-6322 (Print)
IS  - 0001-6322 (Linking)
VI  - 104
IP  - 3
DP  - 2002 Sep
TI  - Distribution of intraneuronal immunoreactivity for the prion protein in human 
      prion diseases.
PG  - 320-6
AB  - Intraneuronal prion protein (PrP) immunoreactivity (INIR), which might represent 
      the non-pathological, cellular form of PrP, needs to be distinguished from 
      disease-associated deposits specific for prion disease (PrD). In adjacent 
      sections of PrD and control brains we applied pretreatments, one of which 
      enhances the immunoreactivity of disease-associated PrP, and another that 
      enhances INIR. We observed an inverse correlation between the proportion of 
      neurons with INIR and the intensity of disease-associated PrP immunoreactivity 
      and severity of lesions. Additionally, we found large intracytoplasmic 
      inclusion-like bodies in ballooned neurons in PrD cases. We noted that the 3F4 
      (epitope: amino acids 109-112) anti-PrP antibody labels more INIR than antibodies 
      directed against amino acids 23-85 (BG4) or 140-180 (KG9) in PrD cases, in 
      contrast to controls, but all antibodies immunolabel more INIR in PrD brains. The 
      up-regulation of PrP might represent an early loss of function of the 
      non-pathological form of PrP, in parallel with a neurotoxic effect of 
      accumulating disease-associated isoform, as part of the pathogenesis of prion 
      diseases.
FAU - Kovacs, Gabor G
AU  - Kovacs GG
AD  - Institute of Neurology, University of Vienna, AKH 4J, Wahringer Gurtel 18-20, POB 
      48, 1097 Vienna, Austria.
FAU - Voigtlander, Till
AU  - Voigtlander T
FAU - Hainfellner, Johannes A
AU  - Hainfellner JA
FAU - Budka, Herbert
AU  - Budka H
LA  - eng
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
DEP - 20020508
PL  - Germany
TA  - Acta Neuropathol
JT  - Acta neuropathologica
JID - 0412041
RN  - 0 (PrPC Proteins)
RN  - 0 (PrPSc Proteins)
SB  - IM
MH  - Adult
MH  - Aged
MH  - Brain/metabolism/pathology
MH  - Creutzfeldt-Jakob Syndrome/etiology/*metabolism/*pathology
MH  - Humans
MH  - Immunohistochemistry
MH  - Middle Aged
MH  - Neurons/*metabolism
MH  - PrPC Proteins/*biosynthesis
MH  - PrPSc Proteins/*biosynthesis
EDAT- 2002/08/13 10:00
MHDA- 2002/10/18 04:00
CRDT- 2002/08/13 10:00
PHST- 2001/08/16 00:00 [received]
PHST- 2002/03/22 00:00 [revised]
PHST- 2002/03/22 00:00 [accepted]
PHST- 2002/08/13 10:00 [pubmed]
PHST- 2002/10/18 04:00 [medline]
PHST- 2002/08/13 10:00 [entrez]
AID - 10.1007/s00401-002-0550-8 [doi]
PST - ppublish
SO  - Acta Neuropathol. 2002 Sep;104(3):320-6. doi: 10.1007/s00401-002-0550-8. Epub 
      2002 May 8.