PMID- 20561933
OWN - NLM
STAT- MEDLINE
DCOM- 20100927
LR  - 20211020
IS  - 1872-7972 (Electronic)
IS  - 0304-3940 (Print)
IS  - 0304-3940 (Linking)
VI  - 479
IP  - 3
DP  - 2010 Aug 2
TI  - Omega-3 and omega-6 fatty acids suppress ER- and oxidative stress in cultured 
      neurons and neuronal progenitor cells from mice lacking PPT1.
PG  - 292-6
LID - 10.1016/j.neulet.2010.05.083 [doi]
AB  - Reactive oxygen species (ROS) damage brain lipids, carbohydrates, proteins, as 
      well as DNA and may contribute to neurodegeneration. We previously reported that 
      ER- and oxidative stress cause neuronal apoptosis in infantile neuronal ceroid 
      lipofuscinosis (INCL), a lethal neurodegenerative storage disease, caused by 
      palmitoyl-protein thioesterase-1 (PPT1) deficiency. Polyunsaturated fatty acids 
      (PUFA) are essential components of cell membrane phospholipids in the brain and 
      excessive ROS may cause oxidative damage of PUFA leading to neuronal death. Using 
      cultured neurons and neuroprogenitor cells from mice lacking Ppt1, which mimic 
      INCL, we demonstrate that Ppt1-deficient neurons and neuroprogenitor cells 
      contain high levels of ROS, which may cause peroxidation of PUFA and render them 
      incapable of providing protection against oxidative stress. We tested whether 
      treatment of these cells with omega-3 or omega-6 PUFA protects the neurons and 
      neuroprogenitor cells from oxidative stress and suppress apoptosis. We report 
      here that both omega-3 and omega-6 fatty acids protect the Ppt1-deficient cells 
      from ER- as well as oxidative stress and suppress apoptosis. Our results suggest 
      that PUFA supplementation may have neuroprotective effects in INCL.
CI  - Published by Elsevier Ireland Ltd.
FAU - Kim, Sung-Jo
AU  - Kim SJ
AD  - Section on Developmental Genetics, Program on Developmental Endocrinology and 
      Genetics, Eunice Kennedy Shriver National Institute of Child Health and Human 
      Development, NIH, Bethesda, MD 20892-1830, United States.
FAU - Zhang, Zhongjian
AU  - Zhang Z
FAU - Saha, Arjun
AU  - Saha A
FAU - Sarkar, Chinmoy
AU  - Sarkar C
FAU - Zhao, Zhenwen
AU  - Zhao Z
FAU - Xu, Yan
AU  - Xu Y
FAU - Mukherjee, Anil B
AU  - Mukherjee AB
LA  - eng
GR  - Z01 HD000910-29/ImNIH/Intramural NIH HHS/United States
GR  - BB_/Biotechnology and Biological Sciences Research Council/United Kingdom
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
DEP - 20100604
PL  - Ireland
TA  - Neurosci Lett
JT  - Neuroscience letters
JID - 7600130
RN  - 0 (Fatty Acids, Omega-3)
RN  - 0 (Fatty Acids, Omega-6)
RN  - 0 (Reactive Oxygen Species)
RN  - EC 3.1.2.- (Thiolester Hydrolases)
RN  - EC 3.1.2.22 (palmitoyl-protein thioesterase)
SB  - IM
MH  - Animals
MH  - Apoptosis/drug effects
MH  - Brain/cytology
MH  - Cells, Cultured
MH  - Endoplasmic Reticulum/*drug effects/physiology
MH  - Fatty Acids, Omega-3/*pharmacology
MH  - Fatty Acids, Omega-6/*pharmacology
MH  - Mice
MH  - Mice, Knockout
MH  - Neuronal Ceroid-Lipofuscinoses/genetics/metabolism
MH  - Neurons/cytology/*drug effects/metabolism
MH  - Oxidative Stress/*drug effects
MH  - Reactive Oxygen Species/metabolism
MH  - Stem Cells/cytology/*drug effects/metabolism
MH  - Thiolester Hydrolases/*genetics
PMC - PMC2904481
MID - NIHMS217389
EDAT- 2010/06/22 06:00
MHDA- 2010/09/29 06:00
PMCR- 2011/08/01
CRDT- 2010/06/22 06:00
PHST- 2010/03/26 00:00 [received]
PHST- 2010/05/12 00:00 [revised]
PHST- 2010/05/26 00:00 [accepted]
PHST- 2010/06/22 06:00 [entrez]
PHST- 2010/06/22 06:00 [pubmed]
PHST- 2010/09/29 06:00 [medline]
PHST- 2011/08/01 00:00 [pmc-release]
AID - S0304-3940(10)00712-3 [pii]
AID - 10.1016/j.neulet.2010.05.083 [doi]
PST - ppublish
SO  - Neurosci Lett. 2010 Aug 2;479(3):292-6. doi: 10.1016/j.neulet.2010.05.083. Epub 
      2010 Jun 4.