PMID- 18948101
OWN - NLM
STAT- MEDLINE
DCOM- 20090105
LR  - 20191003
IS  - 0014-5793 (Print)
IS  - 0014-5793 (Linking)
VI  - 582
IP  - 27
DP  - 2008 Nov 12
TI  - RAGE signaling contributes to neuroinflammation in infantile neuronal ceroid 
      lipofuscinosis.
PG  - 3823-31
LID - 10.1016/j.febslet.2008.10.015 [doi]
AB  - Palmitoyl-protein thioesterase-1 (PPT1) deficiency causes infantile neuronal 
      ceroid lipofuscinosis (INCL), a devastating childhood neurodegenerative storage 
      disorder. We previously reported that neuronal apoptosis in INCL is mediated by 
      endoplasmic reticulum-stress. ER-stress disrupts Ca(2+)-homeostasis and 
      stimulates the expression of Ca(2+)-binding proteins. We report here that in the 
      PPT1-deficient human and mouse brain the levels of S100B, a Ca(2+)-binding 
      protein, and its receptor, RAGE (receptor for advanced glycation end-products) 
      are elevated. We further demonstrate that activation of RAGE signaling in 
      astroglial cells mediates pro-inflammatory cytokine production, which is 
      inhibited by SiRNA-mediated suppression of RAGE expression. We propose that RAGE 
      signaling contributes to neuroinflammation in INCL.
FAU - Saha, Arjun
AU  - Saha A
AD  - Section on Developmental Genetics, Program on Developmental Endocrinology and 
      Genetics, Eunice Kennedy Shriver National Institute of Child Health and Human 
      Development, National Institutes of Health, Bethesda, MD 20892-1830, United 
      States.
FAU - Kim, Sung-Jo
AU  - Kim SJ
FAU - Zhang, Zhongjian
AU  - Zhang Z
FAU - Lee, Yi-Ching
AU  - Lee YC
FAU - Sarkar, Chinmoy
AU  - Sarkar C
FAU - Tsai, Pei-Chih
AU  - Tsai PC
FAU - Mukherjee, Anil B
AU  - Mukherjee AB
LA  - eng
GR  - Z99 HD999999/Intramural NIH HHS/United States
PT  - Journal Article
DEP - 20081021
PL  - England
TA  - FEBS Lett
JT  - FEBS letters
JID - 0155157
RN  - 0 (Cytokines)
RN  - 0 (NF-kappa B)
RN  - 0 (Nerve Growth Factors)
RN  - 0 (RNA, Small Interfering)
RN  - 0 (Receptor for Advanced Glycation End Products)
RN  - 0 (Receptors, Immunologic)
RN  - 0 (S100 Calcium Binding Protein beta Subunit)
RN  - 0 (S100 Proteins)
RN  - 0 (S100B protein, human)
RN  - 0 (S100b protein, mouse)
RN  - EC 2.7.10.2 (src-Family Kinases)
RN  - EC 2.7.12.2 (Mitogen-Activated Protein Kinase Kinases)
RN  - EC 3.1.2.- (Thiolester Hydrolases)
RN  - EC 3.1.2.22 (palmitoyl-protein thioesterase)
SB  - IM
MH  - Animals
MH  - Apoptosis
MH  - Astrocytes/metabolism/pathology
MH  - Brain/immunology/pathology
MH  - Cytokines/biosynthesis
MH  - Encephalitis/genetics/*immunology/*pathology
MH  - Humans
MH  - Mice
MH  - Mice, Knockout
MH  - Mitogen-Activated Protein Kinase Kinases/metabolism
MH  - NF-kappa B/metabolism
MH  - Nerve Growth Factors/metabolism
MH  - Neuronal Ceroid-Lipofuscinoses/genetics/*immunology/*pathology
MH  - RNA, Small Interfering/genetics
MH  - Receptor for Advanced Glycation End Products
MH  - Receptors, Immunologic/agonists/genetics/*metabolism
MH  - S100 Calcium Binding Protein beta Subunit
MH  - S100 Proteins/metabolism
MH  - Signal Transduction
MH  - Thiolester Hydrolases/genetics
MH  - src-Family Kinases/metabolism
PMC - PMC2597448
MID - NIHMS78641
EDAT- 2008/10/25 09:00
MHDA- 2009/01/06 09:00
PMCR- 2009/11/12
CRDT- 2008/10/25 09:00
PHST- 2008/09/17 00:00 [received]
PHST- 2008/10/06 00:00 [revised]
PHST- 2008/10/09 00:00 [accepted]
PHST- 2008/10/25 09:00 [pubmed]
PHST- 2009/01/06 09:00 [medline]
PHST- 2008/10/25 09:00 [entrez]
PHST- 2009/11/12 00:00 [pmc-release]
AID - S0014-5793(08)00830-2 [pii]
AID - 10.1016/j.febslet.2008.10.015 [doi]
PST - ppublish
SO  - FEBS Lett. 2008 Nov 12;582(27):3823-31. doi: 10.1016/j.febslet.2008.10.015. Epub 
      2008 Oct 21.