PMID- 28901522
OWN - NLM
STAT- MEDLINE
DCOM- 20180604
LR  - 20180604
IS  - 1791-3004 (Electronic)
IS  - 1791-2997 (Linking)
VI  - 16
IP  - 5
DP  - 2017 Nov
TI  - Glutathione system in Wolfram syndrome 1‑deficient mice.
PG  - 7092-7097
LID - 10.3892/mmr.2017.7419 [doi]
AB  - Wolfram syndrome 1 (WS) is a rare neurodegenerative disease that is caused by 
      mutations in the Wolfram syndrome 1 (WFS1) gene, which encodes the endoplasmic 
      reticulum (ER) glycoprotein wolframin. The pathophysiology of WS is ER stress, 
      which is generally considered to induce oxidative stress. As WS has a 
      well‑defined monogenetic origin and a model for chronic ER stress, the present 
      study aimed to characterize how glutathione (GSH), a major intracellular 
      antioxidant, was related to the disease and its progression. The concentration of 
      GSH and the activities of reduction/oxidation system enzymes GSH peroxidase and 
      GSH reductase were measured in Wfs1‑deficient mice. The GSH content was lower in 
      most of the studied tissues, and the activities of antioxidative enzymes varied 
      between the heart, kidneys and liver tissues. The results indicated that GSH may 
      be needed for ER stress control; however, chronic ER stress from the genetic 
      syndrome eventually depletes the cellular GSH pool and leads to increased 
      oxidative stress.
FAU - Porosk, Rando
AU  - Porosk R
AD  - Department of Biochemistry, Institute of Biomedicine and Translational Medicine, 
      University of Tartu, 50411 Tartu, Estonia.
FAU - Kilk, Kalle
AU  - Kilk K
AD  - Department of Biochemistry, Institute of Biomedicine and Translational Medicine, 
      University of Tartu, 50411 Tartu, Estonia.
FAU - Mahlapuu, Riina
AU  - Mahlapuu R
AD  - Department of Biochemistry, Institute of Biomedicine and Translational Medicine, 
      University of Tartu, 50411 Tartu, Estonia.
FAU - Terasmaa, Anton
AU  - Terasmaa A
AD  - Centre of Excellence for Genomics and Translational Medicine, Institute of 
      Biomedicine and Translational Medicine, University of Tartu, 50411 Tartu, 
      Estonia.
FAU - Soomets, Ursel
AU  - Soomets U
AD  - Department of Biochemistry, Institute of Biomedicine and Translational Medicine, 
      University of Tartu, 50411 Tartu, Estonia.
LA  - eng
PT  - Journal Article
DEP - 20170831
PL  - Greece
TA  - Mol Med Rep
JT  - Molecular medicine reports
JID - 101475259
RN  - 0 (Membrane Proteins)
RN  - 0 (wolframin protein)
RN  - EC 1.11.1.9 (Glutathione Peroxidase)
RN  - EC 1.8.1.7 (Glutathione Reductase)
RN  - GAN16C9B8O (Glutathione)
SB  - IM
MH  - Aging
MH  - Animals
MH  - Endoplasmic Reticulum Stress
MH  - Glutathione/*metabolism
MH  - Glutathione Peroxidase/metabolism
MH  - Glutathione Reductase/metabolism
MH  - Kidney/enzymology
MH  - Liver/enzymology
MH  - Male
MH  - Membrane Proteins/deficiency/*genetics/metabolism
MH  - Mice
MH  - Mice, Inbred C57BL
MH  - Mice, Knockout
MH  - Myocardium/enzymology
MH  - Pancreas/enzymology
MH  - Wolfram Syndrome/genetics/*pathology
EDAT- 2017/09/14 06:00
MHDA- 2018/06/05 06:00
CRDT- 2017/09/14 06:00
PHST- 2017/04/01 00:00 [received]
PHST- 2017/07/27 00:00 [accepted]
PHST- 2017/09/14 06:00 [pubmed]
PHST- 2018/06/05 06:00 [medline]
PHST- 2017/09/14 06:00 [entrez]
AID - 10.3892/mmr.2017.7419 [doi]
PST - ppublish
SO  - Mol Med Rep. 2017 Nov;16(5):7092-7097. doi: 10.3892/mmr.2017.7419. Epub 2017 Aug 
      31.