PMID- 31985491
OWN - NLM
STAT- MEDLINE
DCOM- 20201027
LR  - 20230224
IS  - 1558-8238 (Electronic)
IS  - 0021-9738 (Print)
IS  - 0021-9738 (Linking)
VI  - 130
IP  - 3
DP  - 2020 Mar 2
TI  - Underglycosylated prion protein modulates plaque formation in the brain.
PG  - 1087-1089
LID - 134842 [pii]
LID - 10.1172/JCI134842 [doi]
AB  - The prion agent is unique in biology and is comprised of prion protein scrapie 
      (PrPSc), a self-templating conformational variant of the host encoded prion 
      protein cellular (PrPC). The deposition patterns of PrPSc in the CNS can vary 
      considerably from a diffuse synaptic pattern to large plaque-like aggregates. 
      Alterations of PrPC posttranslational processing can change PrPSc deposition 
      patterns; however, the mechanism underlying these observations is unclear. In 
      this issue of the JCI, Sevillano and authors determined that parenchymal PrPSc 
      plaques of the mouse brain preferentially incorporated underglycosylated PrPC 
      that had been liberated from the cell surface by the metalloproteinase, ADAM-10, 
      in combination with heparan sulfate. These results provide mechanistic insight 
      into the formation of PrPSc plaques and suggest that PrP posttranslational 
      modifications direct pathogenicity as well as the rate of disease progression.
FAU - Bartz, Jason C
AU  - Bartz JC
LA  - eng
GR  - P01 AI077774/AI/NIAID NIH HHS/United States
GR  - R01 NS103763/NS/NINDS NIH HHS/United States
GR  - R01 NS107246/NS/NINDS NIH HHS/United States
PT  - Comment
PT  - Journal Article
PT  - Research Support, N.I.H., Extramural
PT  - Research Support, Non-U.S. Gov't
PL  - United States
TA  - J Clin Invest
JT  - The Journal of clinical investigation
JID - 7802877
RN  - 0 (Polysaccharides)
RN  - 0 (Prion Proteins)
RN  - 0 (Prions)
SB  - IM
CON - J Clin Invest. 2020 Mar 2;130(3):1350-1362. PMID: 31985492
MH  - Animals
MH  - Brain/metabolism
MH  - Mice
MH  - Polysaccharides
MH  - *Prion Diseases/genetics
MH  - Prion Proteins/genetics
MH  - *Prions/genetics/metabolism
PMC - PMC7269559
COIS- Conflict of interest: The author has declared that no conflict of interest 
      exists.
EDAT- 2020/01/28 06:00
MHDA- 2020/10/28 06:00
PMCR- 2021/03/02
CRDT- 2020/01/28 06:00
PHST- 2020/01/28 06:00 [pubmed]
PHST- 2020/10/28 06:00 [medline]
PHST- 2020/01/28 06:00 [entrez]
PHST- 2021/03/02 00:00 [pmc-release]
AID - 134842 [pii]
AID - 10.1172/JCI134842 [doi]
PST - ppublish
SO  - J Clin Invest. 2020 Mar 2;130(3):1087-1089. doi: 10.1172/JCI134842.