PMID- 30760647
OWN - NLM
STAT- MEDLINE
DCOM- 20200415
LR  - 20200415
IS  - 1945-4589 (Electronic)
IS  - 1945-4589 (Linking)
VI  - 11
IP  - 3
DP  - 2019 Feb 13
TI  - Shaoyao Gancao Tang (SG-Tang), a formulated Chinese medicine, reduces aggregation 
      and exerts neuroprotection in spinocerebellar ataxia type 17 (SCA17) cell and 
      mouse models.
PG  - 986-1007
LID - 10.18632/aging.101804 [doi]
AB  - Spinocerebellar ataxia (SCA) type 17 is an autosomal dominant ataxia caused by 
      expanded polyglutamine (polyQ) tract in the TATA-box binding protein (TBP). 
      Substantial studies have shown involvement of compromised mitochondria biogenesis 
      regulator peroxisome proliferator-activated receptor gamma-coactivator 1 alpha 
      (PGC-1alpha), nuclear factor erythroid 2-related factor 2 (NRF2), nuclear factor-Y 
      subunit A (NFYA), and their downstream target genes in the pathogenesis of 
      polyQ-expansion diseases. The extracts of Paeonia lactiflora (P. lactiflora) and 
      Glycyrrhiza uralensis (G. uralensis) have long been used as a Chinese herbal 
      medicine (CHM). Shaoyao Gancao Tang (SG-Tang) is a formulated CHM made of P. 
      lactiflora and G. uralensis at a 1:1 ratio. In the present study, we demonstrated 
      the aggregate-inhibitory and anti-oxidative effect of SG-Tang in 293 TBP/Q(79) 
      cells. We then showed that SG-Tang reduced the aggregates and ameliorated the 
      neurite outgrowth deficits in TBP/Q(79) SH-SY5Y cells. SG-Tang upregulated 
      expression levels of NFYA, PGC-1alpha, NRF2, and their downstream target genes in 
      TBP/Q(79) SH-SY5Y cells. Knock down of NFYA, PGC-1alpha, and NRF2 attenuated the 
      neurite outgrowth promoting effect of SG-Tang on TBP/Q(79) SH-SY5Y cells. 
      Furthermore, SG-Tang inhibited aggregation and rescued motor-deficits in SCA17 
      mouse model. The study results suggest the potential of SG-Tang in treating SCA17 
      and probable other polyQ diseases.
FAU - Chen, Chiung-Mei
AU  - Chen CM
AD  - Department of Neurology, Chang-Gung Memorial Hospital, Chang-Gung University 
      College of Medicine, Taoyuan 33305, Taiwan.
FAU - Chen, Wan-Ling
AU  - Chen WL
AD  - Department of Neurology, Chang-Gung Memorial Hospital, Chang-Gung University 
      College of Medicine, Taoyuan 33305, Taiwan.
FAU - Hung, Chen-Ting
AU  - Hung CT
AD  - Department of Life Science, National Taiwan Normal University, Taipei 11677, 
      Taiwan.
FAU - Lin, Te-Hsien
AU  - Lin TH
AD  - Department of Life Science, National Taiwan Normal University, Taipei 11677, 
      Taiwan.
FAU - Lee, Ming-Chung
AU  - Lee MC
AD  - Brion Research Institute, New Taipei City 23143, Taiwan.
FAU - Chen, I-Cheng
AU  - Chen IC
AD  - Department of Neurology, Chang-Gung Memorial Hospital, Chang-Gung University 
      College of Medicine, Taoyuan 33305, Taiwan.
FAU - Lin, Chih-Hsin
AU  - Lin CH
AD  - Department of Neurology, Chang-Gung Memorial Hospital, Chang-Gung University 
      College of Medicine, Taoyuan 33305, Taiwan.
FAU - Chao, Chih-Ying
AU  - Chao CY
AD  - Department of Neurology, Chang-Gung Memorial Hospital, Chang-Gung University 
      College of Medicine, Taoyuan 33305, Taiwan.
FAU - Wu, Yih-Ru
AU  - Wu YR
AD  - Department of Neurology, Chang-Gung Memorial Hospital, Chang-Gung University 
      College of Medicine, Taoyuan 33305, Taiwan.
FAU - Chang, Kuo-Hsuan
AU  - Chang KH
AD  - Department of Neurology, Chang-Gung Memorial Hospital, Chang-Gung University 
      College of Medicine, Taoyuan 33305, Taiwan.
FAU - Hsieh-Li, Hsiu Mei
AU  - Hsieh-Li HM
AD  - Department of Life Science, National Taiwan Normal University, Taipei 11677, 
      Taiwan.
FAU - Lee-Chen, Guey-Jen
AU  - Lee-Chen GJ
AD  - Department of Life Science, National Taiwan Normal University, Taipei 11677, 
      Taiwan.
LA  - eng
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
PL  - United States
TA  - Aging (Albany NY)
JT  - Aging
JID - 101508617
RN  - 0 (CCAAT-Binding Factor)
RN  - 0 (Drugs, Chinese Herbal)
RN  - 0 (NF-E2-Related Factor 2)
RN  - 0 (NFE2L2 protein, human)
RN  - 0 (NFYA protein, human)
RN  - 0 (Peptides)
RN  - 0 (Peroxisome Proliferator-Activated Receptor Gamma Coactivator 1-alpha)
RN  - 0 (TATA-Box Binding Protein)
RN  - 0 (jackyakamcho-tang)
RN  - 26700-71-0 (polyglutamine)
RN  - Spinocerebellar Ataxia 17
SB  - IM
MH  - Animals
MH  - CCAAT-Binding Factor/genetics/metabolism
MH  - Cell Line
MH  - Drug Evaluation, Preclinical
MH  - Drugs, Chinese Herbal/*pharmacology/*therapeutic use
MH  - Gene Knockdown Techniques
MH  - Glycyrrhiza uralensis
MH  - Humans
MH  - Mice, Transgenic
MH  - Molecular Targeted Therapy
MH  - NF-E2-Related Factor 2/genetics/metabolism
MH  - Neuronal Outgrowth/drug effects
MH  - Oxidative Stress/drug effects
MH  - Paeonia
MH  - Peptides/metabolism
MH  - Peroxisome Proliferator-Activated Receptor Gamma Coactivator 
      1-alpha/genetics/metabolism
MH  - Phytotherapy
MH  - Spinocerebellar Ataxias/*drug therapy/metabolism
MH  - TATA-Box Binding Protein/drug effects/metabolism
PMC - PMC6382417
OTO - NOTNLM
OT  - NFYA
OT  - NRF2
OT  - PGC-1alpha
OT  - Shaoyao Gancao Tang
OT  - oxidative stress
OT  - polyQ aggregates
OT  - spinocerebellar ataxia 17/TBP
COIS- CONFLICTS OF INTEREST: The authors declare that there are no conflicts of 
      interest.
EDAT- 2019/02/15 06:00
MHDA- 2020/04/16 06:00
PMCR- 2019/02/15
CRDT- 2019/02/15 06:00
PHST- 2018/11/03 00:00 [received]
PHST- 2019/01/24 00:00 [accepted]
PHST- 2019/02/15 06:00 [pubmed]
PHST- 2020/04/16 06:00 [medline]
PHST- 2019/02/15 06:00 [entrez]
PHST- 2019/02/15 00:00 [pmc-release]
AID - 101804 [pii]
AID - 10.18632/aging.101804 [doi]
PST - ppublish
SO  - Aging (Albany NY). 2019 Feb 13;11(3):986-1007. doi: 10.18632/aging.101804.