PMID- 12138171
OWN - NLM
STAT- MEDLINE
DCOM- 20021113
LR  - 20210206
IS  - 0021-9258 (Print)
IS  - 0021-9258 (Linking)
VI  - 277
IP  - 39
DP  - 2002 Sep 27
TI  - Molecular basis of scrapie strain glycoform variation.
PG  - 36775-81
AB  - Transmissible spongiform encephalopathies (TSE) are characterized by the 
      conversion of a protease-sensitive host glycoprotein, prion protein or PrP-sen, 
      to a protease-resistant form (PrP-res). PrP-res molecules that accumulate in the 
      brain and lymphoreticular system of the host consist of three differentially 
      glycosylated forms. Analysis of the relative amounts of the PrP-res glycoforms 
      has been used to discriminate TSE strains and has become increasingly important 
      in the differential diagnosis of human TSEs. However, the molecular basis of 
      PrP-res glycoform variation between different TSE agents is unknown. Here we 
      report that PrP-res itself can dictate strain-specific PrP-res glycoforms. The 
      final PrP-res glycoform pattern, however, can be influenced by the cell and 
      significantly altered by subtle changes in the glycosylation state of PrP-sen. 
      Thus, strain-specific PrP-res glycosylation profiles are likely the consequence 
      of a complex interaction between PrP-res, PrP-sen, and the cell and may indicate 
      the cellular compartment in which the strain-specific formation of PrP-res 
      occurs.
FAU - Vorberg, Ina
AU  - Vorberg I
AD  - Laboratory of Persistent Viral Diseases, NIAID, National Institutes of Health, 
      Rocky Mountain Laboratories, Hamilton, Montana 59840, USA.
FAU - Priola, Suzette A
AU  - Priola SA
LA  - eng
PT  - Journal Article
DEP - 20020723
PL  - United States
TA  - J Biol Chem
JT  - The Journal of biological chemistry
JID - 2985121R
RN  - 0 (PrPSc Proteins)
SB  - IM
MH  - Animals
MH  - Blotting, Western
MH  - Brain/metabolism
MH  - Cell-Free System
MH  - *Glycosylation
MH  - Humans
MH  - Mice
MH  - Phenotype
MH  - PrPSc Proteins/*chemistry/metabolism
MH  - Prion Diseases/*metabolism
MH  - Scrapie/*metabolism
MH  - Tumor Cells, Cultured
EDAT- 2002/07/26 10:00
MHDA- 2002/11/26 04:00
CRDT- 2002/07/26 10:00
PHST- 2002/07/26 10:00 [pubmed]
PHST- 2002/11/26 04:00 [medline]
PHST- 2002/07/26 10:00 [entrez]
AID - S0021-9258(18)36660-2 [pii]
AID - 10.1074/jbc.M206865200 [doi]
PST - ppublish
SO  - J Biol Chem. 2002 Sep 27;277(39):36775-81. doi: 10.1074/jbc.M206865200. Epub 2002 
      Jul 23.