PMID- 21771318
OWN - NLM
STAT- PubMed-not-MEDLINE
DCOM- 20111110
LR  - 20211020
IS  - 1750-1326 (Electronic)
IS  - 1750-1326 (Linking)
VI  - 6
IP  - 1
DP  - 2011 Jul 19
TI  - Peroxisome proliferator activator receptor gamma coactivator-1alpha (PGC-1alpha) 
      improves motor performance and survival in a mouse model of amyotrophic lateral 
      sclerosis.
PG  - 51
LID - 10.1186/1750-1326-6-51 [doi]
AB  - BACKGROUND: Amyotrophic lateral sclerosis (ALS) is a devastating 
      neurodegenerative disease that affects spinal cord and cortical motor neurons. An 
      increasing amount of evidence suggests that mitochondrial dysfunction contributes 
      to motor neuron death in ALS. Peroxisome proliferator-activated receptor gamma 
      co-activator-1alpha (PGC-1alpha) is a principal regulator of mitochondrial biogenesis and 
      oxidative metabolism. RESULTS: In this study, we examined whether PGC-1alpha plays a 
      protective role in ALS by using a double transgenic mouse model where PGC-1alpha is 
      over-expressed in an SOD1 transgenic mouse (TgSOD1-G93A/PGC-1alpha). Our results 
      indicate that PGC-1alpha significantly improves motor function and survival of 
      SOD1-G93A mice. The behavioral improvements were accompanied by reduced blood 
      glucose level and by protection of motor neuron loss, restoration of 
      mitochondrial electron transport chain activities and inhibition of stress 
      signaling in the spinal cord. CONCLUSION: Our results demonstrate that PGC-1alpha 
      plays a beneficial role in a mouse model of ALS, suggesting that PGC-1alpha may be a 
      potential therapeutic target for ALS therapy.
FAU - Zhao, Wei
AU  - Zhao W
AD  - Department of Neurology, Mount Sinai School of Medicine, New York, NY 10029, USA. 
      giulio.pasinetti@mssm.edu.
FAU - Varghese, Merina
AU  - Varghese M
FAU - Yemul, Shrishailam
AU  - Yemul S
FAU - Pan, Yong
AU  - Pan Y
FAU - Cheng, Alice
AU  - Cheng A
FAU - Marano, Paul
AU  - Marano P
FAU - Hassan, Sadiq
AU  - Hassan S
FAU - Vempati, Prashant
AU  - Vempati P
FAU - Chen, Fei
AU  - Chen F
FAU - Qian, Xianjuan
AU  - Qian X
FAU - Pasinetti, Giulio M
AU  - Pasinetti GM
LA  - eng
PT  - Journal Article
DEP - 20110719
PL  - England
TA  - Mol Neurodegener
JT  - Molecular neurodegeneration
JID - 101266600
PMC - PMC3156746
EDAT- 2011/07/21 06:00
MHDA- 2011/07/21 06:01
PMCR- 2011/07/19
CRDT- 2011/07/21 06:00
PHST- 2010/11/23 00:00 [received]
PHST- 2011/07/19 00:00 [accepted]
PHST- 2011/07/21 06:00 [entrez]
PHST- 2011/07/21 06:00 [pubmed]
PHST- 2011/07/21 06:01 [medline]
PHST- 2011/07/19 00:00 [pmc-release]
AID - 1750-1326-6-51 [pii]
AID - 10.1186/1750-1326-6-51 [doi]
PST - epublish
SO  - Mol Neurodegener. 2011 Jul 19;6(1):51. doi: 10.1186/1750-1326-6-51.